Pediatric_Swallowing_and_Feeding_Assessment_and_Management,_Third.pdf

Pediatric
Swallowing and Feeding
Assessment and Management
Third Edition

Pediatric
Assessment and Management
Third Edition
Joan C. Arvedson, PhD
Linda Brodsky, MD
Maureen A. Lefton-Greif, PhD

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Library of Congress Cataloging-in-Publication Data
Names: Arvedson, Joan C., author, editor. | Brodsky, Linda, editor. |
Lefton-Greif, Maureen A., author, editor.
Title: Pediatric swallowing and feeding : assessment and management / Joan C.
Arvedson, Linda Brodsky, Maureen A. Lefton-Greif.
Description: Third edition. | San Diego, CA : Plural Publishing, [2020] |
Includes bibliographical references and index.
Identifiers: LCCN 2019013064| ISBN 9781944883515 (alk. paper) | ISBN
1944883517 (alk. paper)
Subjects: | MESH: Feeding and Eating Disorders of Childhood | Deglutition
Disorders | Feeding Behavior—physiology | Deglutition—physiology |
Infant | Child
Classification: LCC RJ463.I54 | NLM WM 175 | DDC 618.92/31—dc23
LC record available at https://lccn.loc.gov/2019013064
Disclaimer: Please note that ancillary content (such as documents, audio, and video,
etc.) may not be included as published in the original print version of this book.

v
Contents
Foreword vii
Preface ix
About the Editors xi
Contributors xiii
1 Overview of Diagnosis and Treatment 1
Joan C. Arvedson and Maureen A. Lefton-Greif
2 Anatomy, Embryology, Physiology, and Normal Development 11
3 Neurodevelopmental Assessment of Swallowing and Feeding 75
Brian Rogers and Shannon M. Theis
4 The Upper Airway and Swallowing 149
Robert Chun and Margaret L. Skinner
5 Pediatric Gastroenterology 191
Ellen L. Blank
6 Pediatric Nutrition 237
Mary Beth Feuling and Praveen S. Goday
7 Clinical Swallowing and Feeding Assessment 261
Joan C. Arvedson, Maureen A. Lefton-Greif, and Donna J. Reigstad
8 Instrumental Evaluation of Swallowing 331
Maureen A. Lefton-Greif, Joan C. Arvedson, Robert Chun, and
David C. Gregg
9 Management of Swallowing and Feeding Disorders 369
10 Pulmonary Manifestations and Management Considerations 453
for Aspiration
J. Michael Collaco and Sharon A. McGrath-Morrow

vi Pediatric Swallowing and Feeding: Assessment and Management
11 Drooling and Saliva/Secretion Management 479
12 Clinical Genetics: Evaluation and Management of Patients With 517
Craniofacial Anomalies Associated With Feeding Disorders
Julie E. Hoover-Fong and Natalie M. Beck
13 Behavioral Feeding Disorders: Etiologies, Manifestations, and 551
Management
Meghan A. Wall and Alan H. Silverman
Index 577

vii
Foreword
It has been 25 years since the first edition
of this landmark publication Pediatric
Swallowing and Feeding: Assessment and
Management was published. The second,
updated edition was published in 2002.
Now, in 2020, we have the third edition
of this fundamental text concerning the
understanding and care of pediatric swal-
lowing and feeding. The editors, one of
whom unfortunately was deceased before
publication, have recognized the advances
and changes in the understanding of the
information now available for the care of
pediatric swallowing and feeding chal-
lenges. They have recruited an outstanding
group of contributors for this newest edition
and there are numerous critically important
updates and additions. The editors have
included the World Health Organization’s
International Classification of Functioning,
Disability, and Health as the functional
basis for all areas of the book. This text is
important as there are an increased num-
ber of children with complex medical and
health care conditions who are at risk for
feeding and swallowing disorders. This
third edition stresses the need for a team
approach and it also documents the use of
“virtual” teams. This is evidenced through
the chapter contributors who are profes-
sionals in their respective fields. Chapter 10
is especially important now as it documents
the pulmonary manifestations and consid-
erations concerning aspiration in pediatric
patients. Chapter 12 addresses the genetics
underlying many of these conditions, which
was information that was unavailable in the
first two editions.
Pediatric Swallowing and Feeding: Assess-
ment and Management, Third Edition is the
fundamental holistic source for all health
care professionals who provide care for chil-
dren with swallowing and feeding problems
throughout the world. The previous editions
have been, and now this updated third edi-
tion continues to be the standard for infor-
mation concerning diagnosis and care of
these children.
Robert J. Ruben, MD, FAAP, FACS
Distinguished University Professor
Departments of Otorhinolaryngology—
Head and Neck Surgery and Pediatrics
Albert Einstein College of Medicine
Montefiore Medical Center
Bronx, New York

ix
Preface
This third edition of Pediatric Swallowing
and Feeding: Assessment and Management,
now co-edited with Maureen A. Lefton-
Greif, PhD, is published at a time when
recognition of the complexities of infants
and children with swallowing and feeding
disorders is increasing. Recent advances
in genetics and epigenetics and the neuro-
physiologic underpinnings of feeding and
swallowing development and their disor-
ders have contributed to the appreciation of
the complicated inter-relationships among
structures, functions, and the environment
throughout childhood. This body of infor-
mation has advanced this field since publi-
cation of the first two editions of this book
in 1993 and 2002. Consequently, this third
edition is long overdue. It includes signifi-
cant updates and considerable new infor-
mation, making it a “new” edition rather
than a simply revised edition.
We trust that this edition meets the
challenges of balancing updates with new
information, while adhering to the salient
and immutable basic concepts that underlie
this area of practice. Notably, breathing and
eating are basic to survival. Their disrup-
tions can lead to significant compromises
in nutrition and growth, respiratory health,
development and academic skills, and
overall general health and well-being. With
medical advances and the increases in the
survival and life expectancy of medically
fragile children, more attention has been
given to the multidisciplinary needs of these
children. Nonetheless, high-quality evi-
dence to support the care of these children
and the development of consensus-driven
guidelines have not kept pace with the rec-
ognition of the needs of these children.
The World Health Organization’s empha-
sis on “function” and “participation” serve
as essential steps in the development of
meaningful evaluations and effective inter-
ventions, and mandates that professionals
set high priorities on interactions between
caregivers and children, and the need for
non-stressful feedings from preterm infants
through teenage years and into adulthood.
Focusing on only “oral skills” or “safe swal-
lowing” is not enough.
This edition builds on the first two in
which Dr. Linda Brodsky contributed her
extraordinary medical knowledge and lead-
ership in many ways. She is missed not only
for her role in this book, but for her con-
tributions to research and patient care in
pediatric otolaryngology. We have built on
her knowledge and passion for children and
their families.
We acknowledge the many people who
made this edition possible. First, we offer
a special thank you to all the authors who
shared their extensive knowledge and expe-
rience in their specialty areas and for their
generous time commitments given their
busy clinical and research schedules.
We thank Beth Ansel, PhD, and Jeanne
Pinto, MA, for their superb editing, sugges-
tions, and attention to detail. The editors
at Plural Publishing have paid attention
to the many details necessary to bring this
book to publication, and we thank them for
their patience and expertise. We are grateful

x Pediatric Swallowing and Feeding: Assessment and Management
for the families who gave permission for
their children to be photographed adding
examples of the real purposes for all of us—
enhancing the lives of children with swal-
lowing and feeding disorders.
Most of all we thank all the families
and caregivers who have trusted us with
the care of their children. We are in awe of
their courage, inspired by their strength,
grateful for their contributions to the care
of future generations of children with swal-
lowing and feeding disorders, and delight
in the joy they have brought to us. Finally,
we thank our families, to whom this book
is dedicated.

xi
About the Editors
Joan C. Arvedson, PhD, is a speech-language
pathologist, with Specialty in Pediatric Feed-
ing and Swallowing Disorders at the Children’s
Hospital of Wisconsin-Milwaukee and a clini-
cal professor in the Department of Pediatrics,
Medical College of Wisconsin. She is recognized
internationally for her clinical work in pediat-
ric swallowing and feeding disorders, lecturing/
teaching, and scientific publications. The first two
editions of this book were published while she
was at the Children’s Hospital of Buffalo/Kaleida
Health in Buffalo, NY. She and Dr. Lefton-Greif
co-authored Pediatric Videofluoroscopic Swallow
Studies: A Professional Manual with Caregiver
Guidelines. Dr. Arvedson developed an online
course, Interpretation of videofluoroscopic swal-
low studies of infants and children: A study guide
to improve diagnostic skills and treatment planning. She also developed independent study
videoconferences for the American Speech-Language-Hearing Association’s professional
development initiatives. Dr. Arvedson is a founding member of the Board of Certified
Specialists in Swallowing and Swallowing Disorders. She is a Fellow of ASHA and was
awarded Honors of the Association in 2016. Dr. Arvedson is a member of the editorial
board of Dysphagia. She is past-president of the New York State Speech-Language-Hearing
Association and the Society for Ear, Nose, and Throat Advances in Children.

xii Pediatric Swallowing and Feeding: Assessment and Management
Linda Brodsky, MD (1952–2014), an interna-
tionally recognized pediatric otolaryngologist,
was Chief of Pediatric Otolaryngology at the Chil-
dren’s Hospital of Buffalo/Kaleida Health in Buf-
falo, New York; Professor at the State University of
New York at Buffalo Medical School; Director of
the Children Hospital’s Center for Pediatric Oto-
laryngology and Communication Disorders. Dr.
Brodsky was co-editor of the first two editions of
Pediatric Swallowing and Feeding: Assessment and
Management with Dr. Arvedson. In 2014, prelimi-
nary discussions were underway for this third
edition. She’s authored more than 100 scientific
papers and 27 book chapters and served on the
editorial boards of several medical journals. She
was listed in the Best Doctors in America series
and Who’s Who in Science and Engineering. Dr.
Brodsky was presented with the Sylvan Stool award for excellence in teaching by the Society
for Ear, Nose, and Throat Advances in Children. She was a strong advocate for mentorship
of young women in medicine. Her devotion to her patients and tenacity in advocating for
their care was legendary. Dr. Brodsky is missed by her family, colleagues, and patients.
Maureen A. Lefton-Greif, PhD, is Professor in
the Departments of Pediatrics, Otolaryngology—
Head and Neck Surgery, and Physical Medicine
and Rehabilitation at Johns Hopkins Medical
Institutions. She is an internationally recognized
speech-language pathologist for her clinical exper-
tise and research on swallowing and its develop-
ment and disorders in children of all ages. Her
work focuses on optimizing pediatric swallowing
evaluations to facilitate the prompt initiation of
treatment and lessen the consequences associated
with dysphagia. Dr. Lefton-Greif is the recipient
of grants and support from National Institutes of
Health—Deafness and Other Communication
Disorders, Ataxia-Telangiectasia Children’s Proj-
ect, and the Muscular Dystrophy Association. She
and Dr. Arvedson co-authored the book, Pediatric
Videofluoroscopic Swallowing Studies: A Professional Manual with Caregiver Guidelines.
More recently, she and Dr. Bonnie Martin-Harris developed the BaByVFSSImP©. She is a
Fellow of ASHA and a founding member and the first vice-president of the Board of Certified
Specialists in Swallowing and Swallowing Disorders. Dr. Lefton-Greif serves on the editorial
advisory boards of Dysphagia and the Canadian Journal of Speech-Language Pathology.

xiii
Contributors
Joan C. Arvedson, PhD, CCC-SLP, BCS-S
Board Certified Specialist in Swallowing
and Swallowing Disorders
Program Coordinator, Feeding and
Swallowing Services
Children’s Hospital of
Wisconsin-Milwaukee
Milwaukee, Wisconsin
Chapters 1, 2, 7, 8, 9, and 11
Natalie M. Beck, MGC, CGC
Genetic Counselor
Johns Hopkins McKusick-Nathans
Institute of Genetic Medicine
Baltimore, Maryland
Chapter 12
Ellen L. Blank, MD, MA
Retired Pediatric Gastroenterologist
Children’s Hospital of Wisconsin
Associate Adjunct Professor of
Pediatrics-Bioethics
Medical College of Wisconsin
Chapter 5
Robert Chun, MD
Associate Professor
Division of Pediatric Otolaryngology
Department of Otolaryngology
Chapters 4 and 8
J. Michael Collaco, MD, MS, MBA,
MPH, PhD
Associate Professor
Johns Hopkins University School of
Medicine
Eudowood Division of Pediatric
Respiratory Sciences
Baltimore, Maryland
Chapter 10
Mary Beth Feuling, MS, RD, CSP, CD
Advanced Practice Dietitian
Clinical Nutrition
Chapter 6
Praveen S. Goday, MBBS, CNSC, FAAP
Professor of Pediatrics
Division of Pediatric Gastroenterology
and Nutrition
Chapter 6
David C. Gregg, MD
Medical Direction Pediatric Imaging
Associate Professor of Radiology
Chapter 8
Julie E. Hoover-Fong, MD, PhD
Associate Professor
McKusick-Nathans Institute of Genetic
Medicine
Greenberg Center for Skeletal Dysplasias
Johns Hopkins University
Baltimore, Maryland
Chapter 12

xiv Pediatric Swallowing and Feeding: Assessment and Management
Maureen A. Lefton-Greif, PhD,
CCC-SLP, BCS-S
Professor of Pediatrics, Otolaryngology—
Head and Neck Surgery, and Physical
Medicine and Rehabilitation
Eudowood Division of Pediatric
Respiratory Sciences
Medicine
Baltimore, Maryland
Chapters 1, 2, 7, 8, 9, and 11
Sharon A. McGrath-Morrow, MD,
MBA
Division of Pediatric Pulmonary
Johns Hopkins School of Medicine
Baltimore, Maryland
Chapter 10
Donna J. Reigstad, MS, OTR/L
Senior Occupational Therapist
Feeding Disorders Program
Kennedy Krieger Institute
Baltimore, Maryland
Chapters 7 and 9
Brian Rogers, MD
Institute on Development and Disability
Department of Pediatrics
Oregon Health and Science University
Portland, Oregon
Chapter 3
Alan H. Silverman, PhD
Pediatric Psychologist
Chapter 13
Margaret L. Skinner, MD
Assistant Professor, Pediatric
Otolaryngology and Pediatrics
Director, Multidisciplinary Pediatric
Aerodigestive Center
Medicine
Baltimore, Maryland
Chapter 4
Shannon M. Theis, PhD, CCC-SLP
Assistant Professor
Department of Pediatrics
Department of Otolaryngology—Head
and Neck Surgery
School of Medicine
Oregon Health and Science University
Adjunct Faculty, Portland State University
Portland, Oregon
Chapter 3
Meghan A. Wall, PhD, BCBA
Child and Adolescent Psychologist
Assistant Clinical Professor of Psychiatry
Chapter 13

To Linda Brodsky for all she has contributed in the past and how she continues
to influence professionals who follow in her footsteps. We miss you.
To my family: Sons and daughters-in-law Stephen and
Tara, Mark and Julie, along with grandsons Matthew,
Jonathan, and Jason. You are all very special to me.
To my husband Geoffrey, daughters and sons-in-law Jennifer and Daniel,
Alissa and Daniel, and grandchildren Madelyn, Alexander, Emily,
and Cooper. I love you and am grateful to share my life with you.

1
1Overview of Diagnosis
and Treatment
Introduction
During the years since the second edition
of this book, there has been an exponen-
tial increase in basic and clinical research
related to swallowing and feeding in infants
and children. The complexities of interact-
ing systems continue to present challenges
to clinicians and to parents. All involved in
the care of children strive to help them to
be healthy and to grow appropriately, while
ensuring that eating and drinking are plea-
surable with no stress to children or their
caregivers. Factors that have not changed
relate to basic physiologic functions.
Breathing and eating are the most
basic physiologic functions defining the
beginning of life for newborn infants out-
side of the womb. Breathing is reflexive,
life sustaining, and occurs in response to
the transition from the fluid environment
of the womb to the postnatal air environ-
ment. Eating is partly instinctual and partly
a learned response. Eating requires the
ingestion of nutrients provided by an out-
side source. In the newborn infant, sucking
and swallowing require a complex series of
events and coordination of the neurologic,
respiratory, and gastrointestinal (GI) sys-
tems. Normal GI function must occur in
digestion of foods to provide nutrients. All
of these functions are mediated by the integ-
rity of physical and emotional maturation.
The act of feeding is a dyadic process
that requires interaction between the feeder,
usually the mother, and the infant. From
the beginning, feeding should be parent led
with emphasis on quality of feeding, and not
on volume, which often results in stressful
feedings and a potentially reduced volume
of intake and refusals. The pleasure of eating
extends beyond the feeling of satiety to the
pleasure gained through food ingested by
the infant and provided by the mother, who
is most often the primary caregiver. This
interactive primary relationship is the first
for every neonate. It serves as a foundation
for normal development, somatic growth,
communication skills, and psychosocial
well-being. Thus, feeding of the newborn
infant, young child, and rapidly growing
teen is an activity with far-reaching con-
sequences. When feeding is disrupted, the
sequelae can include malnutrition, behav-
ioral abnormalities, and severe distress
for family and child alike. Interruption
of growth and development sometimes
cannot be reversed if it occurs at a critical
time during the early months and years of a
child’s life (Chapter 3). Lifelong disabilities
may result.

2 Pediatric Swallowing and Feeding: Assessment and Management
Prevalence
Currently, more than 100,000 newborn
infants are given diagnoses of feeding prob-
lems after being discharged from acute care
hospitals, and more than one-half mil-
lion children (3–17 years) in the United
States are diagnosed with dysphagia annu-
ally (Bhattacharyya, 2015; CDC/NCHS
National Hospital Discharge Survey, 2010).
The number of children with swallowing
and feeding disorders has been increasing
in part due to recent medical and techno-
logical advances, which have improved the
survival of many infants and children who
previously would not have survived. The
range and complexity of their problems
will continue to challenge the health care,
educational, and habilitation/rehabilitation
systems because many of these children are
now living longer, remaining healthier, and
having greater expectations for leading full
and productive lives.
Approximately 40% of children born
preterm have swallowing/feeding disorders.
Globally, an estimated 15 million infants are
born preterm (less than 37 weeks’ gestation),
and the number is increasing (World Health
Organization [WHO], 2017). Although
many children and their families have ben-
efited greatly, the increasing number of chil-
dren born prematurely at low birth weight
(less than 2,500 g), very low birth weight (less
than 1,500 g), and extremely low birth weight
(less than 600 g) are frequently confronted
with multiple complex medical problems.
In comparison to full-term infants, late
preterm infants (34-0/7 to 36-6/7 weeks
gestation) are at increased risk for respira-
tory and neurologic complications that may
produce or exacerbate feeding difficulties
(Engle, Tomashek, & Wallman, 2007; Mally,
Bailey, & Hendricks-Munoz, 2010). Other
infants with genetic, cardiac, and gastroin-
testinal abnormalities are faced with com-
plex medical and in some instances surgical
problems. Early recognition and interven-
tion have been invaluable despite the cog-
nitive disabilities, cerebral palsy, chronic
pulmonary problems, structural deficits,
and neurologic impairments that infants
endure. Swallowing and feeding problems
compound most of these conditions.
Developmental
Considerations
After the establishment of adequate respi-
ration and physiologic stability, the highest
priority for caregivers is to meet the nutri-
tional needs of their newborn infants. To
achieve this goal successfully, infants and
children of all ages require a well-func-
tioning oral sensorimotor and swallow-
ing mechanism, overall adequate health
(including respiratory, gastrointestinal, and
neurologic), appropriate nutrition, central
nervous system integration, and adequate
musculoskeletal tone.
In addition, the emergence of commu-
nication, an often-overlooked process, is
closely aligned with successful swallowing
and feeding, particularly in young children
(Malas, Trudeau, Chagnon, & McFarland,
2015). Normal feeding patterns are reflected
in the early developmental pathways that
sequentially and rapidly emerge during the
first several months and years of life. Com-
munication is one of the most important
of those pathways. The interrelationship
between feeding, shared by all biologic crea-
tures, and language-based, verbal commu-
nication, unique to humans, cannot be over-
emphasized. The comparative anatomy of
the upper aerodigestive tract and its impli-

1. OVERVIEW OF Diagnosis and Treatment 3
cation for the development of human com-
munication has been established (e.g., Lait-
man & Reidenberg, 1993, 2013; LaMantia et
al., 2016; Lieberman, McCarthy, Hiiemae, &
Palmer 2001; Madriples & Laitman, 1987).
Children who are born prematurely with
very low birth weight or neurologic im-
pairment are commonly found to have swal-
lowing and feeding problems. Other high-
risk children are those experiencing birth
trauma, prenatal and perinatal asphyxia,
and a multitude of genetic syndromes with
accompanying structural and neurologic
impairment (Chapters 3 and 12). The pres-
ence of cardiac, pulmonary, and GI disease
often creates additional difficulty in sorting
out primary and secondary etiologies. Diag-
nosis and management in these patients
present even greater challenges (Table 1–1).
The ability to feed an infant successfully
and thereby nurture an infant is imprinted
early on the maternal–infant relationship.
Normal oral sensorimotor development in-
cludes the establishment of (a) stability and
mobility of the ingestive system, (b) rhyth-
micity, (c) sensation, and (d) oral-motor
efficiency and economy (Gisel, Birnbaum,
& Schwartz, 1998). Optimally, maternal, as
well as paternal, and infant bonding begins
at the outset by providing nutrition with
Table 1–1. Major Diagnostic Categories Associated With Swallowing and Feeding
Disorders in Infants and Children
Neurologic Encephalopathies (e.g., cerebral palsy, perinatal asphyxia)
Traumatic brain injury
Neoplasms
Intellectual disability
Developmental delay
Anatomic and
structural
Congenital (e.g., tracheoesophageal fistula and esophageal
atresia, cleft palate)
Acquired (e.g., tracheostomy, vocal fold paralysis or paresis)
Genetic Chromosomal (e.g., Down syndrome)
Syndromic (e.g., Pierre Robin sequence, Treacher Collins
syndrome, CHARGE syndrome)
Inborn errors of metabolism
Secondary to
systemic illness
Respiratory (e.g., bronchopulmonary dysplasia, chronic lung
disease of prematurity, bronchopulmonary dysplasia)
Gastrointestinal (e.g., inflammatory conditions, GI
dysmotility, constipation)
Congenital cardiac anomalies
Psychosocial
and behavioral
Oral deprivation
Secondary to unresolved or resolved medical condition
Iatrogenic

visual and auditory stimulation of loving
and concerned parents. Thus, swallowing
and feeding disorders likely have negative
impact not only on the physical but also on
the psychosocial well-being of the infant
and child with caregivers.
Sensorimotor Function
The epidemiology of oral sensorimotor dys-
function in the general population and in
the population of children with neurologic
impairments is not well defined. Precise
incidence and prevalence data are difficult
to ascertain. Cerebral palsy (CP) serves
as an example of the range of estimates
that continue to be similar from multiple
sources that have reported approximately
20% to 85% of children with CP are believed
to have swallowing difficulties at some time
during their lives (Benfer, Weir, Bell, Ware,
Davies, & Boyd, 2013; Parkes, Hill, Platt, &
Donnelly, 2010). During the first year of life
of all children with CP, 57% are estimated to
have problems with sucking, 38% with swal-
lowing, and 33% with malnutrition (Reilly,
Skuse, & Poblete, 1996). As the severity of
CP increases, not surprisingly the sever-
ity of the oral sensorimotor dysfunction
increases. The most severely affected are
children with spastic quadriparesis, 90% of
whom have swallowing and feeding prob-
lems (Benfer et al., 2013; Paulson & Vargus-
Adams, 2017; Stallings, Charney, Davies, &
Cronk, 1993). During the first five years
of life, the overall incidence of dysphagia
decreases in children with CP and par-
ticularly in those with better baseline and
improving gross motor function (Benfer,
Weir, Bell, Ware, Davies, & Boyd, 2017 ).
These findings suggest that gross motor
skills and their improvement may herald
those at risk for “persistent” dysphagia.
Team Approaches
to Swallowing/
Feeding Disorders
Feeding disorders that may or may not
include swallowing deficits (dysphagia)
manifest in many different ways. Resistance
to accepting foods, lack of energy for the
work of oral feeding, and oral sensorimotor
disabilities broadly encompass most prob-
lems (Gisel et al., 1998; Kerzner, Milano,
MacLean, Berall, Stuart, & Chatoor, 2015).
Effective management of these medically
complex children depends on the expertise
of many specialists working independently
and as a team (Chapter 9). A few examples
follow, not intended to be an inclusive list,
since different institutions and professionals
within those institutions, carry out patient
care in multiple ways. Some teams may spe-
cialize in specific underlying etiologies or
presentations, for example, Aerodigestive
Clinic, Foregut Clinic (focused specifically
on children with tracheoesophageal fistula
and esophageal atresia (TEF/EA), Tracheos-
tomy/Ventilator Clinic, Craniofacial Team
with a subspecialty clinic for those children
with feeding disorders. Team approaches
also may differ depending on availability of
resources that may even include “virtual”
teams. It is important that teams can offer
coordinated consultation and problem-
solving for co-occurring etiologies and
interrelated problems. Essential compo-
nents can be incorporated in all types of
teams (Table 1–2). The family’s ability to
synthesize and cope with multiple, some-
times disparate opinions must also be a top
priority. Whenever possible, an interdis-
ciplinary team model is encouraged. This
approach refers to interaction of a group of
professionals who meet in person with fam-
ily allowing for optimal efficient communi-
cation. Regardless of the type of team, each

professional brings expertise that is useful in
the solution of complex medical problems.
A group philosophy for both evaluation and
treatment engenders respect for other team
members’ expertise. An organized structure
with a clearly defined leader is important.
Finally, a shared fund of knowledge is criti-
cal and results in creative problem-solving
and fruitful research. In situations where
interdisciplinary teams are not possible,
professionals are urged to develop strate-
gies that promote effective communication
with parents and other primary caregivers.
Team member roles are similar regardless of
the specific type team, with all profession-
als providing services within their scope of
practice and training. Most importantly,
parents/caregivers are integral members of
any team.
Over the past 20 years, there has been
increased recognition of the complex inter-
face between feeding disorders and swal-
lowing impairments in children. The term
feeding disorder refers to inappropriate
development of oral intake and its associ-
ated medical, nutritional, and psychosocial
consequences. Swallowing impairments are
more specific to the process of deglutition.
Hence, all children with swallowing impair-
ments have feeding disorders, but not all
children with feeding disorders have swal-
lowing impairments. Importantly, swallow-
ing impairments can lead to the develop-
ment of feeding disorders. Different types
of models and settings have emerged to
accommodate assessment and treatment of
specific patient populations. Some teams
function primarily in an outpatient setting
and serve as a transitional bridge between
inpatient and outpatient settings. Names for
such teams vary and may include the fol-
lowing: Feeding Clinic; Feeding Disorders
Clinic; Nutrition Clinic; or Swallowing,
Feeding, and Nutrition Clinic; and Feeding
and Growing Clinic. Inpatient swallowing
and feeding teams may be separate from
outpatient teams that have different per-
sonnel. Some teams work across in- and
outpatient settings for assessment and man-
agement of children with specific diagnoses
or presentations. Such teams also vary and
may include craniofacial and aerodiges-
tive teams. The core team members usu-
ally include a physician and other health
care providers as dictated by the needs of
the patient population. The primary oral
sensorimotor swallow therapist is most
likely to be a speech-language pathologist,
although in some instances an occupational
therapist may be primary. All teams benefit
from both when underlying knowledge
and experience is extensive with infants and
children demonstrating swallowing and
feeding disorders.
Table 1–2. Essential Components for Successful Feeding Teams
• Collegial interaction among relevant specialists with active
family involvement
• Shared group philosophy for diagnostic approaches and
treatment protocols
• Team leadership with organization for evaluation and
information sharing
• Willingness to engage in creative problem-solving and research
• Time commitment for the labor-intensive nature of such work

Ethical and Legal Challenges
Underlying Care for
Children With Swallowing/
Feeding Disorders
In addition to making evidence-based deci-
sions, all team members must adhere to
the moral and ethical principles within the
framework of their professions as well as
their scopes of practice (Arvedson & Lefton-
Greif, 2007; Horner, Modayil, Chapman, &
Dinh, 2016). Ethics is a discipline that uses
a systematic approach to examine moral-
ity with the intent of promoting the overall
welfare of the community (Lefton-Greif &
Arvedson, 1997). The four primary princi-
ples of ethical decision-making, respect for
autonomy, beneficence, nonmaleficence,
and justice, are reviewed in detail in Beau-
champ and Childress (1994) and Purtilo
(1988). Adherence to these four commit-
ments is critical to decision making that
goes beyond the realm of facts by rendering
judgements. In addition, for pediatrics, deci-
sion making must take into account in “the
child’s best interests.” Bioethics is the disci-
pline that deals with ethical issues that arise
with advances in medicine. Hence, bioethical
dilemmas are not typically defined by pro-
fessional codes of ethics and are often con-
troversial. Bioethical questions may include
issues that range from allocation of resources
(e.g., expensive drugs used in rare diseases)
to stem cell research. As medical advances
continue, it is likely that all professions
involved with children with dysphagia will
be called on to address bioethical quandaries.
Special Considerations
for School, Home, and
Residential Settings
Oral sensorimotor and swallowing special-
ists frequently function outside of a hospi-
tal setting and outpatient clinic. Assessment
and treatment for children with complex
feeding and other medical problems are
common in a variety of educational (school-
based) and residential (home-based) set-
tings. Working knowledge of the challenges
faced by infants and children with a wide
variety of swallowing problems is manda-
tory. Families may be followed through
a center or home-based educational pro-
gram. These services have been mandated
by federal legislation that guarantees a free
and appropriate educational program for all
handicapped children. The Education for
All Handicapped Children Act (1975–1990)
was revised in 1990 and became known as
Individuals with Disabilities Education Act
(IDEA–Public Law No. 94-142). This law
was established to guarantee that all stu-
dents with disabilities are provided with the
same access to public education as students
without disabilities. “IDEA is composed
of four parts, the main two being part A
and part B. Part A covers the general pro-
visions of the law, Part B covers assistance
for education of all children with disabili-
ties, Part C covers infants and toddlers with
disabilities, which includes children from
birth to age three years, and Part D is the
national support programs administered at
the federal level. Each part of the law has
remained largely the same since the origi-
nal enactment in 1975 Individuals with Dis-
abilities Education Act (2017, November
13).” Section 504 of the Rehabilitation Act
of 1973, as amended (Section 504), clari-
fied information about the Americans with
Disabilities Act (ADA, 2008) in the areas of
public elementary and secondary education
(U.S. Department of Education, 2015). The
ADA (2008) broadened the interpretation
of disability, which clearly includes eating.
Schools are bound by IDEA and 504 because
of their responsibility to provide a free and
appropriate public education (FAPE).

Challenges in Caring for
Feeding Disorders
A comprehensive approach to children with
swallowing and oral sensorimotor func-
tion problems can be hampered by the lack
of a shared fund of knowledge. A clearly
defined set of terms related to this rapidly
expanding field is necessary. Several terms
will be defined here with others defined as
they are encountered throughout the book.
Deglutition1
is the act of swallowing and is
just one process in the broader context of
feeding. Swallowing refers to the entire act
of deglutition from placement of food and
liquid into the mouth until they enter the
upper esophagus. Sucking, chewing, and
swallowing are three physiologically dis-
tinct processes occurring during deglutition
(Kennedy & Kent, 1985). Estimates of the
frequency of swallowing have ranged from
600 to 1,000 times per day (Lear, Flanagan,
& Moorrees, 1965). The highest frequency is
during food intake, and the lowest is during
sleep. Aside from providing nourishment
and hydration, swallowing accomplishes
other purposes, such as the removal of
saliva and mucous secretions from the oral,
nasal, and pharyngeal cavities. A decrease in
swallowing frequency may be coupled with
oral sensorimotor dysfunction and thereby
may result in severe drooling (Chapter 11).
Feeding is a broad term to encompass
the process for getting food/liquid into the
mouth (https://en.oxforddictionaries.com/
definition/deglutition). Once food and liq-
uid enter the mouth, the process continues
with bolus formation as the initial process
to include sucking and chewing (depending
on the composition of the food or liquid)
that leads to moving food/liquid through
the mouth, into the pharynx for initiation of
swallowing. Dysphagia is a swallowing defi-
cit (https://en.oxforddictionaries.com/defi
nition/dysphagia). Oral sensorimotor func-
tion refers to all aspects of sensory and motor
functions involving the structures in the oral
cavity and pharynx related to swallowing
from the lips until the onset (or initiation) of
the pharyngeal phase of the swallow (Chap-
ter 2). Finally, nutrition is the process by
which all living organisms obtain the food
and nourishment necessary to sustain life
andsupportgrowth(https://en.oxforddiction
aries.com/definition/us/nutrition).
Care for children with swallowing and
feeding disorders requires a broad knowl-
edge base that must be supplemented by
a thoughtful and often creative problem-
solving approach. The steps in this approach
are universal to the diagnosis and treatment
of any medical condition or illness. Their
importance to the approach of a medically
complex child cannot be overemphasized.
Team care is most effective in developing
alternate strategies when normal swallow-
ing is absent and nutrition is severely com-
promised (Table 1–3).
1
The terms swallowing and deglutition have been used interchangeably. The term swallowing will be used
throughout the text, unless distinguishing between these terms is relevant to the text.
Table 1–3. Process Steps for Diagnosis
and Treatment of Pediatric Swallowing
and Feeding Disorders
• Define problem feeding and swallowing
• Identify etiology(ies)
• Determine appropriate diagnostic tests
• Plan approach to patient/family
• Teach about problem, implement
treatment
• Monitor progress
• Evaluate progress (outcomes focused)

Clinical and Research
Updates for the Care of
Feeding Disorders
This third edition provides updated clini-
cal and research findings that have direct
impact on care for infants and children with
swallowing and feeding disorders. Empha-
ses continue to be placed on the critical
importance of a fund of knowledge across
multiple systems that are factors in chil-
dren of all ages and all underlying etiolo-
gies. Clinical approaches are presented and
discussed in ways that readers are expected
to find useful in the evaluation and man-
agement of infants and children with oral
sensorimotor dysfunction and swallowing
problems.
The next several chapters cover infor-
mation that provides a basis for understand-
ing the common problems associated with
swallowing and feeding disorders. Knowl-
edge of anatomy, embryology, physiology,
and pathophysiology of the upper aerodi-
gestive tract is fundamental for the under-
standing of infants and children with a wide
range of swallowing and feeding disorders.
The following chapters focus on neurode-
velopment (normal and abnormal), airway,
gastroenterology, and nutrition. These chap-
ters are followed by a chapter on oral sen-
sorimotor clinical feeding evaluation and
a chapter on instrumental assessment with
primary focus on videofluoroscopic swallow
studies and fiberoptic endoscopic examina-
tion of swallowing. Significant clinical and
research advances over the past 10 years are
highlighted in these chapters as well as the
chapter on decision making regarding man-
agement strategies and intervention.
Chapters that follow cover specific top-
ics including aspiration and saliva/secre-
tion management. The chapter on cranio-
facial anomalies has an entirely new section
focused on the genetic basis of conditions
associated with swallowing/feeding prob-
lems in infants and children with craniofa-
cial anomalies. The final chapter focuses on
children with psychologic and behavioral
problems, often accompanied by sensory
factors, as major components in their feed-
ing disorders. The importance of integrat-
ing these factors that include parent/child
relationships cannot be overstated. Func-
tional outcome is the goal for every child
and family.
Clinical case studies that are found at
the end of most chapters provide concrete
examples of teamwork with varied empha-
ses that encompass the depth and breadth
of pediatric feeding disorders. Evaluation
and treatment approaches are included
where supported by clinical experience and
the scientific literature. Medical, psychoso-
cial, and satisfaction outcomes are reported
when available. Although there are some
reports in recent years, the literature con-
tinues to be sparse in the areas of pediatric
swallowing and feeding in normal develop-
ment as well as disorders.
Strong emphasis continues to be placed
on the importance of making a diagno-
sis based on etiology of disease preceding
treatment. All professionals involved in
assessment and management of infants and
children in both medical and educational
settings must have appropriate knowledge
and training to assess and treat infants and
children with dysphagia and related condi-
tions. All decision-making, communications,
and interactions with families and other pro-
fessionals must be carried out with adher-
ence to the respective professional ethical
codes of conduct. The overall importance of
an appropriate fund of knowledge and shared
experience employing team approaches is
emphasized throughout this third edition as
in the earlier editions of this book.

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11
2Anatomy, Embryology,
Physiology, and Normal
Development
Summary
The human upper aerodigestive tract is the
most complex neuromuscular unit in the
body. It is the intersection of the digestive,
respiratory, and phonatory systems. Normal
swallowing requires precise integration of
the important functions of breathing, eat-
ing, and speaking. A thorough under-
standing of the anatomy, embryology, and
physiology of these systems is necessary to
appreciate the etiology, diagnosis, and treat-
ment of swallowing and feeding disorders in
infants and children.
Attention to functional anatomy pro-
vides a basis for the discussion of clinically
relevant embryologic development. The
physiology of swallowing, with emphasis on
neurophysiology, posture, and muscle tone,
is presented in detail in this chapter. The
challenges of developmental change begin-
ning with premature infants and extend-
ing through adolescents are nowhere more
apparent than for swallowing and feeding.
Swallowing and feeding are explained in
the context of normal oral sensorimotor
development of the infant and child. Special
focus on the anatomy and physiology of the
airway and gastrointestinal (GI) tract will
help to enhance the reader’s understanding
of the clinical manifestations, diagnosis, and
treatment of swallowing and feeding prob-
lems in children.
Introduction
Deglutition, more commonly referred to as
swallowing,1
is defined as the semiautomatic
motor action of the muscles of the respira-
tory and GI tracts that propels food from the
oral cavity into the stomach (Miller, 1986).
Swallowing functions not only to transport
food to the stomach, but also in clearing the
mouth and pharynx of secretions, mucus,
and regurgitated stomach contents. Thus,
the function of swallowing is nutritive as
well as protective of the lower airways.
The act of swallowing is complex
because respiration, swallowing, and pho-
nation all occur at one anatomic location—
the region of the pharynx and larynx. To
1
The common usage term, swallowing, is used throughout this textbook for ease of reading. Similarly,
ingestion, the taking in of food, will be referred to as feeding or eating (as age appropriate) throughout.

be successful, normal swallowing requires
the coordination of 31 muscles, six cranial
nerves, and multiple levels of the central
nervous system (CNS), including the brain
stem and cerebral cortex (Bosma, 1986).
Thus, understanding the anatomy, embry-
ology, physiology, and normal development
of this functional neuromuscular unit is of
paramount importance to the proper diag-
nosis and treatment of swallowing and feed-
ing disorders in children.
Anatomy
The upper aerodigestive tract consists of
the nose, oral cavity, pharynx, larynx, and
esophagus. The trachea, bronchi, and pul-
monary parenchyma are considered the
lower airways. The upper digestive tract
ends at the entrance to the stomach. Each
area is discussed separately.
Nose
The nose is important for respiration
throughout life, but particularly in neo-
nates (first 28 days of life) and young infants
(up to 6 months), when preferential nasal
breathing is present. The nose also cleans,
warms, and humidifies inspired air. As the
nasal passage continues posteriorly, it opens
at the bilateral posterior nasal choanae into
the nasopharynx, which is an important
anatomic chamber that serves as a resona-
tor for speech production. In addition, the
nasopharynx is one of the two airway con-
duits into the hypopharynx (Figure 2–1).
The lateral nasal walls are composed of
three bones covered with a highly sensitive
INFANT
Tongue
Maxilla
Mandible
Hyoid
Larynx
Trachea
Tongue
Esophagus
Epiglottis
Hypopharynx
Nasopharynx
Vallecula
Soft Palate
Hard Palate
Figure 2–1. Lateral view of the infant’s upper aerodigestive tract. Structures and
boundaries of the oral cavity, pharynx, and larynx are noted.The soft palate is in
close approximation to the valleculae. This anatomic proximity effectively sepa-
rates the oral route for ingestion from the preferred nasal route for respiration.

2. Anatomy, Embryology, Physiology, and Normal Development 13
mucosa—the nasal turbinates. The nose is
separated into two nasal cavities by the mid-
line septum, which is cartilage anteriorly
and bone posteriorly. Septal deviation in
the newborn may occur from birth trauma
and result in severe nasal obstruction lead-
ing to perinatal feeding difficulties (Emami,
Brodsky, Pizzuto, 1996). Other etiologies
of nasal obstruction include, but are not
limited to, choanal atresia, encephalocele,
glioma, nasal dermoid, nasolacrimal duct
cyst, pyriform aperture stenosis, and rhini-
tis (Gnagi Schraff, 2013; see Chapter 4).
Soft palate elevation and retraction seal off
the nasal cavity from the oropharynx and
the oral cavity.
Oral Cavity (Mouth)
The oral cavity is involved in ingestion of
food, vocalization, and oral respiration.
Structures include lips, mandible, maxilla,
floor of the mouth, cheeks, tongue, hard
palate, soft palate, and anterior surfaces of
the anterior tonsillar pillars. Older infants
and children also have teeth for chewing.
The lateral sulci are spaces between the
mandible or maxilla and the cheeks. The
anterior sulci are spaces between the man-
dible or maxilla and the lip muscles.
The structures in the mouth are impor-
tant for bolus formation and oral transit
(described in detail in the following text). In
infancy, the cheeks with fat pads or sucking
pads are important for sucking. The tongue
has attachments to the mandible, hyoid
bone, and styloid process of the cranium by
the extrinsic muscles of the tongue (genio-
glossus, hypoglossus, and styloglossus
muscles) (Bosma, 1972). When anatomic
defects of the lips, palate, maxilla, mandi-
ble, cheeks, or tongue are present, normal
sucking and swallowing may be compro-
mised (see Chapters 4 and 12). In children
with oral sensorimotor problems, food or
liquid can be lodged in both the anterior
and lateral sulci, making bolus preparation
difficult. Muscles involved in bolus forma-
tion and oral transit include the digastric,
palatoglossus, genioglossus, styloglossus,
geniohyoid, mylohyoid, buccinators, and
those muscles intrinsic to the tongue (no
bony attachment, classified by orientation
of the muscle fibers: longitudinal, vertical,
and transverse). Cranial nerves involved
include V, VII, IX, X, XI, and XII (Bosma,
1986; Derkay Schechter, 1998; Perlman
Christensen, 1997).
Pharynx
The pharynx consists of three anatomic
areas (Figures 2–1 and 2–2): the nasophar-
ynx, the oropharynx, and the hypopharynx.
In the infant, the nasopharynx and hypo-
pharynx blend into one structure, and thus
there is no true oropharynx as seen in the
older child. The nasopharynx begins at the
nasal choanae and ends at the elevated soft
palate. The eustachian tubes originate in the
nasopharynx (Bosma, 1967).
As growth and development occur, two
important anatomic changes emerge: (a) the
angle of the nasopharynx at the skull base
becomes more acute and approaches 90°,
and (b) the pharynx elongates so that an
oropharynx is created. The faucial arches
form a bridge between the mouth and the
oropharynx. This junction and the tongue
base form the anterior boundary of the
oropharynx, which extends inferiorly to
the epiglottis. The oropharynx includes
the epiglottis and the valleculae. The val-
leculae are bilateral pockets formed by
the base of the tongue and the epiglottis
(Donner, Bosma, Robertson, 1985). The
hypopharynx (sometimes called the laryn-
geal pharynx) extends from the base of the

epiglottis to the cricopharyngeal muscles in
the upper esophageal sphincter. The ante-
rior wall of the hypopharynx includes the
laryngeal inlet and the cricoid cartilage.
The pyriform sinuses are pockets lateral
and just below the inlet to the larynx. The
vertical enlargement of this space enables
the development of human speech. Phona-
tion of a wide variety of speech sounds can
thus occur. However, this elongation chal-
lenges the timing and coordination needed
for functional swallowing and breathing as
a common and enlarged intersection of the
respiratory and digestive tracts is created
(Laitman Reidenberg, 1993).
The walls of the pharynx consist of three
pairs of constrictor muscles—the superior,
medial, and inferior constrictors. These
striated muscle fibers arise from a median
raphe in the midline of the posterior pha-
ryngeal wall. They extend laterally and
attach to bony and soft tissue structures
located anteriorly. Initiation of the pharyn-
geal swallow function is under voluntary
neural control and becomes involuntary
for completion of the pharyngeal swallow.
This function is under the control of cranial
nerves (CN) V, IX, and X that synapse in the
swallowing center located in the medulla.
Nasopharynx
The nasopharynx is a boxlike structure
located at the base of the skull. It connects
the nasal cavity above with the orophar-
ynx below, and serves as a conduit for air,
OLDER CHILD
Nasopharynx
Oropharynx
Hypopharynx
Larynx
Esophagus
Trachea
Epiglottis
Hyoid
Vallecula
Soft palate Tongue
Figure 2–2. Lateral view of the older child’s upper aerodigestive tract. Note
the wide distance between the soft palate and the larynx. The elongated phar-
ynx is unique to humans and has allowed the development of human speech
production.

a drainage area for the nose and paranasal
sinuses and eustachian tube/middle ear
complex, and a resonator for speech pro-
duction. The boundaries of the nasophar-
ynx are the posterior nasal choanae (anteri-
orly), the soft palate (anterior-inferior), the
skull base (posteriorly), and the hypophar-
ynx in infants and oropharynx in children
and adults (inferiorly). Tongue propulsion
moves a bolus posteriorly and thus assists
in the elevation of the soft palate and closes
off the nasopharynx from the rest of the
pharynx. Anatomic or functional defects of
the soft palate may result in nasopharyngeal
backflow/reflux during oral feedings (Chap-
ters 4 and 12).
The adenoid is a mass of lymphatic tissue
located behind the nasal cavity, in the roof
of the nasopharynx where the nose blends
into the throat. The adenoid, unlike the pala-
tine tonsils, has pseudostratified epithelium.
The adenoid is part of the “Waldeyer ring” of
lymphoid tissue, which includes the palatine
tonsils and the lingual tonsils.
During the first years of life, the adenoid
increases in size. Involution begins at about
age 8 years and extends through puberty.
Excessive enlargement of the adenoid may
cause nasal obstruction and feeding diffi-
culties, even in older children.
Oropharynx
The oropharynx is the posterior extension
of the oral cavity. The oropharynx begins at
the posterior surface of the anterior tonsillar
pillars and extends to the posterior pharyn-
geal wall. The palatine tonsils are attached
to the lateral pharyngeal walls between the
anterior and posterior tonsillar pillars. The
superior boundary of the oropharynx is par-
allel to the pharyngeal aspect of the soft pal-
ate in a line extending back to the posterior
pharyngeal wall. The inferior boundary of
the oropharynx is at the base of the tongue
and includes the epiglottis and valleculae.
The valleculae are wedge-shaped spaces at
the base of the tongue and the epiglottis.
The lingual tonsil is along the tongue base.
When the lingual tonsil becomes enlarged,
it can encroach on the valleculae and cause
significant airway, feeding, and swallow-
ing problems. Enlargement may be seen
when severe gastroesophageal reflux disease
(GERD)/extra-esophageal reflux disease
(EERD)2
is present. The lateral and poste-
rior walls of the oropharynx are formed by
the middle and part of the inferior pharyn-
geal constrictor muscles. The greater cornua
of the hyoid bone are included in the lateral
pharyngeal walls (Donner et al., 1985).
The body of the hyoid bone, located in
the deep musculature of the neck, attaches
to the base of the tongue. The base of the
tongue and the larynx descend inferiorly
during the first 4 years of life. By age 4, the
base of the tongue is anatomically sepa-
rated from the larynx in the vertical plane
and thus becomes the anterior border of the
oropharynx (Caruso Sauerland, 1990).
Because the infant’s larynx is high in the
neck, almost “tucked under” the base of the
tongue, no true oropharynx exists (see Fig-
ures 2–1 and 2–2). Thus, in neonates and
young infants, a single conduit for breath-
ing is created from the nasopharynx to the
hypopharynx that allows them to coordi-
nate sucking, swallowing, and breathing.
2
Gastroesophageal reflux disease (GERD) refers to the abnormal regurgitation of acid into the esophagus
causing symptoms. When acid and other stomach contents emerge from the esophagus into the pharynx,
larynx, mouth, and nasal cavities, the most commonly accepted term is extra-esophageal reflux disease
(EERD) (Sasaki Toohill, 2000).

Hypopharynx
The hypopharynx extends from the base of
the epiglottis at the level of the hyoid bone
down to the cricopharyngeus muscle. Ante-
riorly it ends at the laryngeal inlet above the
true vocal folds at the level of the false vocal
folds and includes the cricoid cartilage. Pos-
teriorly, the hypopharynx ends at the level
of the entrance to the esophagus, which is
guarded by the cricopharyngeus muscle.
This muscle has no median raphe, in con-
trast to the pharyngeal constrictors. Except
during swallowing, belching, or regurgita-
tion, the cricopharyngeus is in a state of
tonic contraction functioning as the pha-
ryngoesophageal sphincter or upper esoph-
ageal sphincter (UES)3
(Caruso Sauer-
land, 1990; Kahrilas et al., 1986). The fibers
of the inferior constrictors attach to the
sides of the thyroid cartilage. These spaces
are known as the pyriform sinuses, and they
extend down to the cricopharyngeus muscle
(Figure 2–3). The oblique fibers of the infe-
rior constrictor muscles end where the hori-
zontal fibers of the cricopharyngeus muscle
3
Terminology is rapidly changing in this field. For purposes of this book, the more familiar term upper
esophageal sphincter (UES) is used.
Figure 2–3. Posterior sketch of the upper aerodigestive tract
(larynx and pharynx). Pathway for food bolus is around the
larynx and down the channels made by the pyriform sinuses,
which elongate during the act of swallowing. The bolus is
moved through the upper esophageal sphincter (UES) par-
tially via action of the hyolaryngeal complex decreasing ten-
sion on the open UES while the larynx is closed and protected
high in the neck under the tongue base.

begin. The lateral and posterior walls of the
hypopharynx are supported by the middle
and inferior constrictors. The anterior
boundary of the hypopharynx is the larynx.
Larynx
The larynx is a complex structure that is the
superior entrance to the trachea. The larynx
consists primarily of cartilages, suspended
by muscle and ligament attachments to the
hyoid bone and cervical vertebrae. The car-
tilages include the epiglottis, thyroid, cri-
coid, and paired arytenoids, cuneiforms,
and corniculates. Intrinsic muscles of the
larynx form the vocal folds (true and false)
that are integral to respiration and pho-
nation. The thyrohyoid and thyrocricoid
ligaments aid in laryngeal suspension and
stability. In order of priority, the three func-
tions of the human larynx are the protection
of the lower airways, respiration, and pho-
nation. The structures important in swal-
low production and in airway protection
during swallowing are described in detail.
Detailed anatomic description of the intrin-
sic muscles of the larynx (involved primar-
ily with phonation) is beyond the scope of
this chapter.
The most important structures of the
larynx that protect against aspiration are
the paired arytenoid cartilages and the two
pairs of vocal folds. In most humans, the
epiglottis plays a role in airway protection.
However, there are examples of children
with congenitally absent epiglottis (Koem-
pel Holinger, 1998) and functional oral
feeding. The epiglottis has a flattened lin-
gual surface, which acts to direct food later-
ally into the recesses formed by the pyriform
sinuses. The movement of food is directed
away from the midline and the laryngeal
inlet. The arytenoid cartilages and the ary-
epiglottic folds, reinforced by the smaller
cuneiform and corniculate cartilages, move
medially to further buttress the larynx from
penetration. The larynx is elevated anteri-
orly under the tongue and mandible by
the hyolaryngeal complex (hyoid bone and
attached musculature).
The valvelike function provided by
the paired false and true vocal folds is the
next and most critical level of laryngeal
structures involved in airway protection.
The false vocal folds (ventricular folds) are
primarily involved in regulating the expira-
tion of air from the lower respiratory tract
(Sasaki Isaacson, 1988). In contrast, the
true vocal folds do not resist expired air but
can prevent inspired air (and foreign mate-
rial) from entering the larynx. Thus, specific
anatomic abnormalities at the laryngeal
level must be precisely defined to avoid seri-
ous sequelae of an incompetent larynx.
Neuroanatomy of the Larynx
Multilevel sphincteric closure of the upper
airway is controlled by the recurrent laryn-
geal nerves. The aryepiglottic folds, made
up of the superior part of the thyroaryte-
noid muscles, approximate to cover the
superior inlet of the larynx. The anterior
gap is protected by the posteriorly displaced
epiglottis, the posterior gap closed by the
arytenoid cartilages (Figure 2–4). The false
vocal folds form the roof of the laryngeal
ventricles and are the second level of protec-
tion within the larynx. The thyroarytenoid
muscles aid in adduction of the false vocal
folds. The third level of protection is the
true vocal folds, with the inferior part of the
thyroarytenoid muscles providing the bulk
of these folds. The true vocal folds attach
to the vocal processes of the arytenoid car-
tilages posteriorly, to the inside surface of
the thyroid lamina laterally, and to the thy-
roid notch anteriorly. Muscular pull by the
arytenoid cartilages controls movement of

the true vocal folds during both swallowing
and phonation.
Innervation of the protective laryn-
geal and respiratory functions is centrally
located in the brain stem. This control relies
on fine sensory and motor innervation to
the region. Sensory innervation of the
supraglottic and glottic areas is provided by
the internal branch of the superior laryngeal
nerve (SLN), a branch of the vagus nerve
(CN X). The recurrent laryngeal nerve
(RLN) (also from CN X) provides sensory
innervation to the subglottic mucosa. The
posterior part of the true vocal folds and the
superior surface of the epiglottis appear to
be the most densely innervated part of the
larynx (Sasaki Isaacson, 1988). Chemi-
cal and thermal receptors are also found in
the supraglottic larynx and are sensitive to
a variety of stimuli. In particular, receptors
sensitive to water in infants and young chil-
dren may explain the favorable response to
cool mist in children with laryngotracheitis,
also known as “croup.” The effect of the mist
slows the rate of respiration while increas-
ing tidal volume, resulting in an overall pos-
itive effect on the respiratory status (Sasaki,
Suzuki, Horiuchi, Kirchner, 1979). Other
sensory receptors of the larynx include joint,
aortic, baroreceptors, and stretch receptors.
These afferent impulses are interpreted at
the brain-stem level in the tractus solitarius.
The ipsilateral RLN (vagus—CN X)
innervates all of the intrinsic muscles of the
larynx except the cricothyroid muscles. The
cricothyroid is innervated by the external
branch of the SLN. Only the interarytenoid
muscles receive bilateral innervation from
the recurrent laryngeal nerves. All of the
intrinsic muscles of the larynx are involved
in adduction except the posterior cricoary-
tenoid muscles, the only abductors of the
vocal folds. Control at the brain-stem level
is within the nucleus ambiguus.
Anatomic changes in the larynx are evi-
dent when SLN paralysis occurs. The lateral
cricoarytenoid muscle, a laryngeal adduc-
tor, rotates the posterior laryngeal commis-
Figure 2–4. Superior view of the larynx showing the intrinsic structures of the larynx.
The laryngeal ventricle is the space between the false and true vocal folds. Airway
closure occurs from distal to proximal regions (i.e., first true vocal folds, next false
vocal folds, and finally aryepiglottic folds).

sure to the paralyzed side. This results in a
foreshortening of the vocal fold on the ipsi-
lateral side, which gives an appearance of
asymmetry or tilt to the larynx. In contrast,
paralysis of the RLN results in a paramedian
position of that vocal fold, caused by the
unopposed adductor action of the ipsilateral
cricothyroid muscle, innervated by an intact
external branch of the SLN.
Esophagus
The esophagus is a muscular tube lined with
mucosa that propels food from the hypo-
pharynx to the stomach. The cricopha-
ryngeus is the major muscle of the upper
esophageal sphincter (UES), also called
the cricopharyngeal sphincter and pharyn-
goesophageal segment (PE segment) and
forms the junction between the hypophar-
ynx and the esophagus. The mucosa just
above the cricopharyngeus muscle is thin
and vulnerable to injury, such as perfora-
tion from foreign bodies (Caruso Sauer-
land, 1990). The gastroesophageal or lower
esophageal sphincter (LES) forms the junc-
tion between the esophagus and the stom-
ach. The LES has transient relaxations in
contrast to the UES which is in tonic con-
traction (discussed later in this chapter).
These sphincters help keep the esophagus
empty between swallows (Derkay Schech-
ter, 1998).
The esophagus is in close proximity to
other structures in the neck and thorax.
In the neck, it lies anterior to the cervical
vertebrae, posterior to the trachea, and
between the carotid arteries. The recurrent
laryngeal nerves are located on either side
of the esophagus in the tracheoesophageal
groove. Other important structures in the
posterior mediastinum related to breathing,
feeding, and swallowing are the left main-
stem bronchus, the aortic arch, the pericar-
dium, and the nerves and blood vessels to
the esophagus.
The wall of the esophagus is composed
of four layers: mucosa, submucosa, mus-
cularis, and adventitia. The mucosa of the
esophagus constitutes three layers of tissue:
epithelium, lamina propria, and muscularis
mucosae. The mucosa of the esophagus is
stratified squamous, continuous with the
epithelium in the pharynx. Intrinsic mus-
cles of the esophagus are found in an outer
longitudinal layer and an inner circular
layer. The posterior and lateral portions of
the longitudinal muscle encircle the inner
muscle layer in a spiral pattern. The upper
third of the esophagus is composed of stri-
ated muscle similar to the constrictors in the
pharynx; the lower two-thirds is made up
of smooth muscle fibers. The pharynx and
proximal esophagus are the only regions in
the body where striated muscle is not under
voluntary neural control. Both sympathetic
and parasympathetic fibers innervate the
esophagus, although the cricopharyngeus
muscle seems to be primarily under para-
sympathetic control via the vagus nerve
(DerkaySchechter,1998).Thevagalmotor
nerve fibers to striated muscles of the upper
esophagus arise from the nucleus ambig-
uus in the brain stem and those to smooth
muscles of originate in the dorsal motor
nucleus, next to the nucleus ambiguus.
This brief description of the esophagus does
not begin to cover the complexities of neu-
ral innervation, muscle types and function,
mucosal changes, connective tissue, and the
extracellular matrix of the esophagus (see
Perlman Konrad Schulze-Delrieu, 1997,
with additional references).
Significantanatomicdifferencesarefound
between the infant and older child/adult (see
Figures 2–1 and 2–2). These differences are
listed by anatomic location in Table 2–1.

Embryology
Embryology is the branch of biology involv-
ing the study of prenatal development that
includes the embryo and the fetus. The anat-
omy of the oral cavity, pharynx, larynx, and
esophagus is the result of embryologic pro-
cesses that begin at fertilization of the ovum
and continue through infancy, childhood,
and even into adulthood. In this section, the
development of the head and neck, respi-
ratory system, digestive system, and perti-
nent parts of the CNS are described in some
detail. However, this section is intended to
provide a brief overview of the develop-
mental processes. Salient features of the
related cardiovascular and musculoskeletal
systems are also reviewed. The interested
student is referred to texts on embryology
for further detail (e.g., Brookes Zietman,
1998; Moore, Persaud, Torchia, 2015;
Table 2–1. Anatomic Locations and Differences Between the Infant’s and Older Child’s
Upper Aerodigestive Tracts
Anatomic
Location
Differences
Infant Older Child
Oral cavity Tongue fills mouth Mouth is larger
Edentulous Dentulous
Tongue rests between lips and sits
against palate
Tongue rests on floor of mouth
Cheeks have sucking pads (fatty
tissue within buccinators)
Tongue rests behind the teeth and is
not against palate
Relatively small mandible Buccinators are muscles for chewing
only
Sulci important in sucking Mandibular-maxillary relationship
relatively normal
Sulci have little functional benefit
Pharynx No definite/distinct oropharynx Elongated pharynx, so distinct
oropharynx exists
Obtuse angle at skull base in
nasopharynx
90º angle at skull base
Larynx One-third adult size Less than one-third true vocal fold of
cartilage
Half true vocal fold of cartilage Flat, wide epiglottis
Narrow, vertical epiglottis By 2 years of age, approximates
adult position re: cervical vertebrae
High in the neck, re: cervical
vertebrae

Schoenwolf, Bleyl, Brauer, Francis-West,
Philippa, 2015). Normal embryologic devel-
opment related to oral sensorimotor func-
tion and swallowing is discussed later in this
chapter, followed by a brief description of
some of the congenital abnormalities that
present with swallowing problems.
Embryonic Period (Weeks 1 to 8)
Human prenatal development begins at fer-
tilization with formation of a zygote. The
zygote is a diploid cell containing 46 chro-
mosomes with half from the mother and
half from the father. Fertilization of the egg
is completed within 24 hours of ovulation.
Repeated mitotic divisions of the zygote
result in a rapid increase in the number of
cells. By the 3rd week, three germ layers
(ectoderm, mesoderm, and endoderm) are
formed from which all tissues and organs
of the embryo develop. The ectoderm gives
rise to the epidermis and the nervous sys-
tem. The mesoderm gives rise to smooth
muscle, connective tissue, and blood vessels.
The endoderm gives rise to the epithelial
linings of respiratory and digestive systems.
During the 3rd week, the CNS and the
cardiovascular system begin to form. The
neural plate, which is the origin of the
CNS, gives rise to the neural folds and the
beginning of the neural tube. The neural
crest consists of neuroectodermal cells that
form a mass between the neural tube and
the overlying surface ectoderm. The neural
crest gives rise to the sensory ganglia of the
cranial and spinal nerves, as well as to sev-
eral skeletal and muscular components in
the head and neck region.
All major organ systems are formed
during the 4th to 8th weeks of development.
During the 4th week, the trilaminar embry-
onic disc forms into a C-shaped cylindrical
embryo, which later becomes the head, tail,
and lateral folds. The dorsal part of the yolk
sac becomes incorporated into the embryo
and gives rise to the primitive gut (Moore et
al., 2015). Infolding at the head region yields
the oropharyngeal membrane. The heart is
carried ventrally, and the developing brain
is at the most cranial part of the embryo. By
the end of the 8th week, the embryo begins
to have a human appearance.
Fetal Period (Week 9 to Birth)
The fetal period begins in the 9th week and
is primarily marked by rapid body growth,
with relatively slower head growth com-
pared with the rest of the body. Differen-
tiation of tissues and organs continues dur-
ing this time. A brief description of major
embryologic changes is followed by more
detailed information regarding systems
directly involved in swallowing.
9 to 12 Weeks
At the beginning of the 9th week, the head
makes up half the length of the fetus, mea-
sured from the crown to the rump (Caruso
Sauerland, 1990). At 9 weeks, the face is
broad, with widely separated eyes, fused
eyelids, and low-set ears. The legs are short
with relatively small thighs. By the end of
12 weeks, the upper limbs will have almost
reached the final relative lengths, although
lower limbs are still slightly shorter than the
final relative lengths.
13 to 16 Weeks
By the 13th week, body length has more
than doubled. Body growth occurs so
rapidly that by the 16th week, the head is
relatively small compared with the end of

the 12th week. Ossification of the skeleton
begins during this period.
17 to 20 Weeks
Somatic growth slows down, but length
continues to increase. Fetal movements are
beginning to be felt by the mother. Eyebrows
and head hair become visible at 20 weeks.
21 to 25 Weeks
Substantial weight gain occurs during this
time. By 24 weeks, the lungs begin produc-
ing surfactant, which is a surface-active
lipid that maintains the patency of the
developing alveoli of the lungs. However,
the respiratory system is still very immature
and unable to sustain life independently. If
born at this premature stage, however, sur-
factant replacement therapy has allowed
some of these premature infants to survive.
26 to 29 Weeks
The lungs are capable of air exchange, but
with some difficulty. The CNS is beginning
to mature, and rhythmic breathing move-
ments are possible although not present in
all infants. Control of body temperature
begins. The eyes are open at the beginning
of this period.
30 to 34 Weeks
By 30 weeks, the pupillary light reflex of
the eyes can be elicited. By 34 weeks, white
fat in the body makes up about 8% of body
weight. The presence of white fat is a devel-
opmental milestone for normal feeding
potential because the infant then begins to
show some nutritional reserves. Body tem-
perature regulation is more stable by 34 to
35 weeks.
35 to 40 Weeks
At 36 weeks, the circumferences of the head
and the abdomen are approximately equal.
After 36 weeks, the abdomen circumfer-
ence may be greater than that of the head.
Although at full term the head is much
smaller relative to the rest of the body than
it was during early fetal life, it is still reason-
ably large in relation to the size of their bod-
ies. The expected time of birth is 38 weeks
after fertilization (gestational age or post-
conceptual age) or 40 weeks after the last
menstrual period. By full term, the amount
of white body fat should be about 16% of
body weight.
Head and Neck Development
Branchial (Pharyngeal)
Apparatus Development
The head and neck are developed from the
branchial apparatus, which consists of bran-
chial arches, pharyngeal pouches, branchial
grooves, and branchial membranes. Bran-
chial arches are derived from the neural
crest cells and begin to develop early in the
4th week, as the neural crest cells migrate
into the future head and neck region. By the
end of the 4th week, four pairs of branchial
arches are visible (Figure 2–5). The fifth
and sixth pairs are too small to be seen
on the surface of the embryo. The bran-
chial arches are separated by the branchial
grooves, which are seen as prominent clefts
in the embryo.
The branchial arches contribute to
formation of the face, neck, nasal cavities,
mouth, larynx, and pharynx, with the mus-
cular components forming striated muscles
in the head and neck. Anatomic develop-
ment of the thyroid and cricoid cartilages

beginning at the 13th week (up to 27 weeks)
reveals a correlation between laryngeal
length and fetal crown-rump (C-R) with no
differences between genders (Gawlikowska-
Stoka et al., 2010). The width of both thy-
roid cartilage laminae was significantly
larger in males than in females across 13 to
27 weeks (Gawlikowska-Stoka et al., 2010)
with similar sexual dysmorphism noted for
glottis opening in postmortem study (Fay-
oux, Marciniak, Deisme, Storme, 2008).
These authors suggest that findings may
be useful in planning treatment of airway
emergencies.
The cranial nerve supply for each bran-
chial arch, along with the skeletal structures
and muscles derived from the branchial
arches are described in Table 2–2.
Facial Development
The mandible is the first structure to form
by the merging of the medial ends of the
two mandibular prominences of the first
branchial arch during the 4th week. Maxil-
lary prominences of the first branchial arch
grow medially toward each other, as do the
medial nasal prominences soon thereaf-
ter. The auricles of the external ear begin
to develop by the end of the 5th week. As
the brain enlarges, a prominent forehead
is noted, the eyes move medially, and the
Heart prominence
Yolk stalk
Body stalk
Otic vesicle
Third branchial arch
Second branchial arch
(Hyoid)
First branchial arch
(Mandibular)
Optic vesicle
Figure 2–5. Human embryo at about 28 days showing early branchial (pharyn-
geal) apparatus relationships. Four pairs of branchial arches can be seen with
their respective branchial grooves.

24
Table 2–2. Cranial Nerves, Structures, and Muscles Derived From Branchial
(Pharyngeal) Arch Components
Arch Cranial Nerves Structures Muscles
First (mandibular) Trigeminal (V) Mandible Muscles of mastication
Maxilla Mylohyoid and anterior
belly of digastric
Malleus, incus Tensor tympani
Zygomatic bone Tensor veli palatini
Temporal bone
(squamous portion)
Second (hyoid) Facial (VII) Stapes Muscles of facial
expression
Styloid process Stapedius
Hyoid bone
(Lesser cornu)
(Upper body)
Stylohyoid
Posterior belly of digastric
Third Glossopharyngeal
(IX)
Hyoid bone
(Greater cornu)
(Inferior body)
Stylopharyngeus
Hypoglossal (XII) Posterior one-third
of tongue
Epiglottis
Fourth and sixth Vagus (X)
SLN
RLN
Tongue
Laryngeal cartilages
Epiglottis (fourth)
Palatoglossus
Cricothyroid
Levator veli palatini
Pharyngeal constrictors
Intrinsic muscles of
larynx
Striated muscles of
esophagus
Note. RLN = recurrent laryngeal nerve; SLN = superior laryngeal nerve.
Source: Adapted from Structures derived from pharyngeal arch components. In K. L. Moore (Ed.), The
developing human (10th ed., p. 160). Philadelphia, PA: Elsevier, 2015.

external ears ascend. At 16 weeks, the eyes
begin to migrate and are situated more ante-
riorly than laterally. The ears are closer to
their final position at the sides of the head.
The medial and lateral nasal promi-
nences are formed by growth of the sur-
rounding mesenchyme, which results in
formation of primitive nasal sacs. The nasal
cavity is separated from the oral cavity by
the oronasal membrane (Figure 2–6), which
ruptures at about 6 weeks. This rupture that
forms the primitive choanae brings the nasal
and oral cavities into direct communication.
If the oronasal membrane does not rupture,
a choanal atresia will make it impossible for
an infant to suck, swallow, and breathe syn-
chronously (Chapter 4). The posterior nasal
choanae are located at the junction of the
nasal cavity and the nasopharynx once the
development of the palate is completed.
Palatal development begins toward the
end of the 5th week and is completed in
the 12th week (Figure 2–7). Development
occurs from anterior to posterior as mes-
enchymal masses merge toward the mid-
line. The primary palate, or medial palatine
process, develops at the end of the 5th week
and is fused by the end of the 6th week to
become the premaxillary part of the max-
illa. The primary palate gives rise to a very
small part of the adult hard palate that is
positioned just posterior (or caudal) to the
incisive foramen of the skull. Subsequently,
the secondary palate develops from two
horizontal lateral palatine processes that
fuse over the course of a few weeks from the
incisive foramen posterior to the soft palate
and uvula. The anterior hard palate (ossi-
fied) is fused by 9 weeks, and the muscular
soft palate is completed by the 12th week.
The nasal septum develops downward
from the merged medial nasal prominences.
During the 9th week, the fusion between the
nasal septum and the palatine processes
begins anteriorly and is completed at the
posterior portion of the soft palate by the
12th week. This process occurs in conjunc-
tion with the fusion of the lateral palatine
processes. The palatine processes fuse about
a week later in female than in male fetuses,
which may explain why isolated cleft palate
is more common in female infants (Burdi,
Mandibular process
Rupturing oronasal membrane
Pharynx
Tongue
Oral cavity
Primary palate
Nasal cavity
Figure 2–6. Sagittal section showing oronasal membrane, which separates
the nasal and oral cavities. At about 6 weeks, the oronasal membrane ruptures
to form the primitive choanae. This brings the nasal and oral cavities into direct
communication.

1969). As the jaws and the neck develop, the
tongue descends and occupies a relatively
smaller space in the oral cavity. The tongue
also develops from the third and fourth
branchial arches.
Prenatal Sucking, Swallowing,
and Breathing Development
The pharyngeal swallow is one of the first
motor responses in the pharynx. It has been
reported between 10 and 14 weeks’ gesta-
tion (Humphry, 1970). Pharyngeal swallows
have been observed in delivered fetuses at
12.5 weeks’ gestation (Humphry, 1970).
Ultrasound studies reveal nonnutritive
suckling/sucking and swallowing in most
fetuses by 15 weeks’ gestation (Moore et
al., 2015). Sucking, suckling, and sucking act
are terms often used interchangeably in the
literature to describe mouthing movements
and ingestion of food by infants (Wolf
Glass, 1992). Suckling, the earliest intake
pattern for liquids, is characterized by a
definite backward and forward movement
of the tongue, with the backward phase
more pronounced (Figure 2–8). In contrast,
sucking begins to emerge at four months of
age, and involves more of an up and down
movement of the tongue and active use of
the lips. A suckling response may be elic-
ited at this stage as noted by the finding that
stroking the lips yields suckling responses
in spontaneously aborted fetuses. True
suckling begins around the 18th to the 24th
week. Self-oral-facial stimulation precedes
suckling and swallowing with consistent
swallowing seen by 22 to 24 weeks’ gesta-
tion (Miller, Sonies, Macedonia, 2003).
Tongue protrusion does not extend beyond
the border of the lips (Morris Klein, 1987).
By the 34th week, most healthy fetuses, if
born at that time, can suckle and swallow
well enough to sustain nutritional needs via
the oral route. Some infants appear coordi-
nated enough to begin oral feedings by 32 to
33 weeks’ gestation (Cagan, 1995).
Infants born late preterm (between 34
0/7 and 36 6/7 weeks of gestation), account
for 70% of all preterm births (Davidoff et al.,
2006; Dong Yu, 2011; Loftin et al., 2010;
Perugu, 2010). The incidence of late pre-
Philtrum
Upper lip
Choanae
Nasal septum
Nostril
Primary palate
(Premaxilla)
Lateral
palatine
process
Figure 2–7. Palatal development from anterior to posterior.The lateral processes fuse
to form most of the hard and soft palate, completed by 9 and 12 weeks, respectively.

term births has increased markedly in the
past two decades with increased prevalence
of medical problems that are also noted in
early term (37 to 38 weeks’ gestation) com-
pared to infants born full term (39 to 41
weeks) (Brown, Speechley, Macnab, Natale,
Campbell, 2014; Hwang et al., 2013; Sahni
Polin, 2013). Feeding difficulties are
reported with high frequency in infants who
are bottle or breastfeeding (Dosani et al.,
2017). There are limited data on feeding
problems in late preterm infants (Bloom-
field et al., 2018; DeMauro, Patel, Medoff-
Cooper, Posencheg, Abbasi, 2011). Gianni
and colleagues (2015) note that nutritional
support is likely to be needed for those late
preterm infants with a birth weight less
than or equal to 2000 g, gestational age of
34 weeks, and born small for gestational age,
develop respiratory distress syndrome, and
require a surgical procedure.
Decreased rates of fetal suckling are
associated with alimentary tract obstruction
or neurologic damage, the latter of which
manifests as intrauterine growth restriction
(Derkay Schechter, 1998). It is estimated
that 450 ml of the total 850 ml of amniotic
fluid produced daily is swallowed in utero
(Bosma, 1986).
Ultrasound has shown that suckling
motions increase in frequency in the later
months of fetal life. The frequency of the
suckling motions can be modified by taste.
Taste buds are evident at 7 weeks’ gestation,
with distinctively mature receptors noted at
12 weeks (Miller, 1982). Ultrasonography is
shown to have a high degree of intra- and
interobserver repeatability for analysis of
sucking and swallowing movements (Levy
et al., 2005).
Digestive System Development
The endoderm of the primitive gut, which
forms in the 4th week, gives rise to most of
the epithelium and glands of the digestive
tract. The muscles, connective tissue, and
other layers comprising the wall of the diges-
tive tract are derived from the splanchnic
mesenchyme (loosely organized connective
tissue) surrounding the endodermal primi-
tive gut. The foregut, midgut, and hindgut
make up the primitive gut.
The derivatives of the foregut include
the pharynx and its derivatives, respira-
tory system, esophagus, stomach, duode-
num (up to the opening of the bile duct),
Figure 2–8. Suckling and sucking comparisons of tongue and mandibular
action. Suckling is characterized by in–out tongue movements and some jaw
opening and closing; sucking is characterized by up–down tongue movements
and less vertical jaw action. Readers are reminded that terms may be used dif-
ferently in the literature.

liver, pancreas, and the biliary apparatus
(gallbladder and biliary duct system). The
celiac artery supplies all derivatives except
the pharynx, respiratory tract, and most of
the esophagus.
The esophagus elongates rapidly and
reaches its final relative length by the 7th
week. If it does not elongate sufficiently,
part of the stomach may be displaced supe-
riorly through the esophageal hiatus in the
thorax, resulting in a congenital hiatal her-
nia (Moore et al., 2015). (See Chapter 5.)
Although the upper third of the esophagus
is made up of striated muscle and the lower
two thirds of smooth or nonstriated muscle,
there is a transition region between the cer-
vical and thoracic levels where striated and
smooth muscle fibers intermingle. Both
types of muscle are innervated by branches
of the vagus nerve (CN X). The esophagus
and airways share common innervations
with complex interrelationships of afferents
and efferents having both sympathetic and
parasympathetic responses, as reviewed by
Jadcherla (2017).
Respiratory System
Development
The respiratory system begins to develop
during the 4th week by formation of a
median laryngotracheal groove in the cau-
dal end of the ventral wall of the primi-
tive pharynx. This laryngotracheal groove
develops into a laryngotracheal diverticu-
lum that then becomes separated from
the primitive pharynx (cranial part of the
foregut) by longitudinal tracheoesophageal
folds. During the 4th and 5th weeks, these
folds fuse and form the tracheoesophageal
septum, which is a partition dividing the
foregut into a ventral and a dorsal portion.
The ventral portion is the laryngotracheal
tube that eventually becomes the larynx,
trachea, bronchi, and lungs. The dorsal
portion becomes the esophagus. It is clear
from these early embryologic changes that
the airway and digestive systems are inextri-
cably related because they initially develop
from the same embryonic structure.
Laryngeal Development
The opening of the laryngotracheal tube
into the pharynx becomes the primitive
glottis. The laryngeal cartilages and muscles
are derived from the 4th and 6th pairs of
branchial arches (see Table 2–2). The epithe-
lium of the mucous membrane lining of the
larynx develops from the endoderm of the
cranial end of the laryngotracheal tube. The
mesenchyme proliferates rapidly at the cra-
nial end of the laryngotracheal tube to pro-
duce paired arytenoid swellings at 5 weeks
(Figure 2–9A). The primitive glottis (Fig-
ure 2–9B), a slitlike opening, is converted
into a T-shaped opening as the arytenoid
swellings grow toward the tongue (Figure
2–9C). This action reduces the developing
laryngeal lumen again to a narrow slit. The
laryngeal lumen is temporarily occluded by
rapid proliferation of the laryngeal epithe-
lium. By the 10th week, recanalization of
the larynx occurs (Figure 2–9D). The epi-
glottis develops from the caudal part of the
hypobranchial eminence. This eminence is
produced by proliferation of mesenchyme
in the ventral parts of the third and fourth
branchial arches.
Tracheobronchial and
Pulmonary Development
The laryngotracheal tube distal to the lar-
ynx gives rise to the epithelium and glands

of the trachea and lungs. The tracheal car-
tilages, connective tissue, and muscles are
derived from the surrounding splanchnic
mesenchyme. The cartilage is in the form
of C-shaped rings in the trachea and major
bronchi. In more peripheral airways, the
cartilage becomes more irregular and less
prominent. The subglottic space is defined
by the cricoid cartilage, the only cartilage
that forms a complete ring. The respira-
tory system develops so that it is capable of
immediate function by full-term gestation.
The lungs must have sufficiently thin alve-
olocapillary membranes and an adequate
amount of surfactant for normal respira-
tion to occur.
Maturation of the lungs occurs in four
periods (Moore et al., 2015):
n Pseudoglandular period (6 to 16 weeks):
Resembles an exocrine gland and
by 16 weeks all major elements have
formed, except those involved with gas
exchange. Respiration is not possible.
n Canalicular period (16 to 26 weeks):
Overlaps with previous period since
cranial segments mature faster than
caudal segments. Lung tissue becomes
highly vascular by the end of this
period. Fetuses born near the end
of this period may survive if given
intensive care, but survival is not always
A B
C D
5 Weeks 6 Weeks
7 Weeks 10 Weeks
Arytenoid
swelling
Arytenoid
swelling
Epiglottis
Primitive glottis
Glottis
Cartilages
Glottis
Epiglottis Epiglottis
Epiglottis
Figure 2–9. Embryologic stages of laryngeal development. A. At 5 weeks, paired arytenoid
swellings develop at cranial end of the laryngotracheal tube. B. At 6 weeks, the primitive glottis
can be seen. C. At 7 weeks, T-shaped opening is evident in the glottis as arytenoid swellings
grow toward the tongue. D. At 10 weeks, recanalization of the larynx occurs.

possible due to respiratory and other
systems still being relatively immature.
n Terminal saccular period (26 weeks to
birth): Many terminal saccules develop,
and their epithelium becomes very
thin. Capillaries bulge into developing
alveoli. The blood–air barrier is
established through intimate contact
between epithelial and endothelial cells
that permit adequate gas exchange
for survival. Complex development of
type I and II alveolar cells takes place.
The type II cells secrete pulmonary
surfactant, which is a monomolecular
film, over the internal walls of the
terminal saccules. That action lowers
surface tension at the air–alveolar inter-
face. Production of surfactant increases
during the final stages of pregnancy,
especially during the last two weeks.
n Alveolar period (32 weeks to 8 years):
Exactly when this period begins
depends on the definition of the
term alveolus. At 32 weeks, saccules
are present and analogous to alveoli.
However, characteristic mature alveoli
do not form until after birth with about
95% of alveoli developing postnatally.
During the first few months after birth,
an exponential increase is seen in the
surface of the air–blood barrier that
is accomplished by multiplication of
alveoli and capillaries. The lungs of
full-term newborn infants contain
about 50 million alveoli (one sixth of
adult number), which make their lungs
denser than adult lungs. By 2 years of
age, most postnatal alveolar develop-
ment is completed (Thurlbeck, 1982).
The lungs are about half-filled with
fluid at birth. Aeration of the lungs
occurs from the rapid replacement of
intra-alveolar fluid by air. The fluid is
cleared by three routes: (a) through
mouth and nose by pressure on the
fetal thorax during delivery, (b) into
the pulmonary capillaries, and (c) into
the lymphatics and pulmonary arteries
and veins. Normal lung development
depends on three factors: (a) adequate
thoracic space for lung growth, (b) fetal
breathing movements, and (c) adequate
amniotic fluid volume (Moore et al.,
2015).
Cardiovascular System
Development
The cardiovascular system is the first organ
system to function in the embryo. By the
end of the 3rd week, blood begins to circu-
late, and the first heartbeat occurs at 21 to
22 days. The heart develops from splanch-
nic mesenchyme as paired endocardial
heart tubes form and fuse into a single heart
tube, which is the primitive heart. From the
4th to the 7th week, the four chambers of
the heart are formed. The critical period of
heart development is from Day 20 to Day
50 after fertilization. The partitioning of the
primitive heart results from complex pro-
cesses, and defects of the cardiac septa are
relatively common.
Fetal blood is oxygenated in the pla-
centa. The lungs are nonfunctional as
organs of respiration during prenatal life.
Adequate respiration in the newborn infant
is dependent on normal circulatory changes
occurring at birth. The modifications that
establish postnatal circulatory patterns at
birth are gradual and continue for the first
several months of life.
Congenital heart disease (CHD) is the
most common cause of major congenital
anomalies, occurring in an estimated 8 per
1,000 live births (van der Linde et al., 2011).
Detection of fetal CHDs is possible as early
as the 17th or 18th week of development.
Although the underlying causes of CHD

need further clarification, single-gene,
chromosomal variations and exposure to
teratogens have been associated with these
problems. See Chapter 12.
Central Nervous System
Development
The CNS develops from the neural plate,
which appears about the middle of the 3rd
week and becomes the neural tube. The cra-
nial end of the neural tube forms the brain,
which consists of the forebrain, midbrain,
and hindbrain. The forebrain is the basis
for the cerebral hemispheres and the dien-
cephalon. The midbrain becomes the adult
midbrain. The hindbrain becomes the pons,
cerebellum, and medulla oblongata. The
spinal cord is formed from the rest of the
neural tube.
The ventricles of the brain and the cen-
tral spinal canal are derived from the lumen
of the neural tube. Proliferation of neuro-
epithelial cells causes the walls of the neu-
ral tube to thicken. These cells give rise to
all nerve and macroglial cells in the CNS.
Twelve pairs of cranial nerves are formed
during the 5th and 6th weeks of develop-
ment. They are classified into three groups
according to their embryological origins:
(a) somatic efferent cranial nerves—troch-
lear (CN IV), abducent (CN VI), hypoglos-
sal (CNXII), and greater part of oculomotor
(CN III); (b) nerves of pharyngeal arches—
trigeminal (CN V), facial (CN VII), glos-
sopharyngeal (IX), and vagus (CN X); and
(c) special sensory nerves—olfactory (CN
I), optic (CN II), and vestibulocochlear (CN
VIII). Neural tube defects are described by
Copp, Stanier, Greene, 2013, but will not
be discussed here.
The cranial nerves of the branchial
arches, described earlier, are particularly
important for normal swallowing. The
CNS regulates the buccal, lingual, and pha-
ryngeal movements necessary for sucking
and swallowing. Four-dimensional ultra-
sound demonstrates that the fetal face is an
important indicator of fetal brain function
at 20 to 24 weeks of gestation, with a range
of facial expressions to include mouthing,
tongue expulsion, and features of emotion-
like behaviors (AboEllail Hata, 2017; Sato
et al., 2014). Further description of CNS
development is found in Chapter 3. The
neural control of deglutition is discussed in
more detail in the physiology section in this
chapter (e.g., Costa, 2018).
Embryologic Abnormalities
Affecting Swallowing
and Feeding
Congenital abnormalities or birth defects
are structuralabnormalitiesofanytype pres-
ent at birth. (See Chapter 12 for a review of
the evaluation and management of patients
with craniofacial anomalies associated with
feeding disorders and an overview of clini-
cally available tests.) Briefly, four clinically
significant types are malformation, disrup-
tion, deformation, and dysplasia. Congeni-
tal abnormalities or malformations result
from both genetic factors and environmen-
tal factors, with some malformations caused
by these factors acting together. An accu-
rate diagnosis is integral for patient care
of children with underlying genetic condi-
tions. Recent advances in sequencing, par-
ticularly whole-exome sequencing (WES),
are identifying genetic basis of disease for
25% to 40% of patients. These percentages
are anticipated to increase as these analyses
become more common (Sawyer et al., 2016).
CNS damage from congenital malfor-
mations is a major underlying cause of swal-
lowing and feeding problems in infants. In

addition, upper airway anomalies or other
anatomic defects may occur. It is estimated
that 5% to 7% of human developmen-
tal abnormalities result from the in utero
action of drugs, viruses, and other environ-
mental factors (Persaud, Chudley, Skalko,
1985). Exposure of the embryo to teratogens
(agents that produce or raise the incidence
of congenital malformations, such as drugs
and viruses) have their effect during the
stage of active differentiation of an organ
or a tissue. The most critical period for
brain development is from 3 to 16 weeks;
however, disruptions in development can
occur after this time period. The brain is
differentiating and growing rapidly at birth
and continues at least throughout the first
2 years of life. Three important principles
must be considered regarding possible sus-
ceptibility to teratogens: (a) critical periods
of development, (b) dosage of the drug or
chemical, and (c) genotype (genetic consti-
tution of the embryo) (Moore et al., 2015).
Injuries early in gestation are generally
more severe for two reasons. First, little
or no barrier exists between blood and
brain, so chemicals enter the brain eas-
ily. After birth, the blood–brain barrier is
more effective. Second, a small injury in the
early developing brain will be magnified by
the effect on the total remaining sequence
of development, which is dependent on
the injured area (Lenn, 1991). Patterns of
malformation occur in recognizable ways
because the parts of the brain that arise from
a region of early injury are malformed after
the injury. Readers interested in brain devel-
opment, early brain injuries, and neuroplas-
ticity are encouraged to review the works
by Anderson, Spencer-Smith, and Wood
(2011); Johnston (2009); Kolb, Harker, and
Gibb (2017); and Staudt (2010).
Low birth weight and prematurity are
other potential complicating factors. Sur-
vival is unlikely with a birth weight of less
than 500 g and a gestational age of less than
22 to 23 weeks. By 28 weeks’ gestational age,
survival is more common because signifi-
cant development occurs in the respira-
tory system and CNS from 24 to 32 weeks.
Detailed descriptions of conditions are
beyond the scope of this chapter. Some
information can be found in separate chap-
ters with direct relevance to swallowing
and feeding factors in infants and children.
Readers are encouraged to keep aware of
updated information available via online
sites, including but not limited to PubMed
(https://www.pubmed.gov) and Online
Mendelian Inheritance in Man (https://
www
.omim.org/). Embryologic abnormali-
ties can affect multiple systems (e.g., CNS,
head and neck structures, respiratory tract,
esophageal and rest of GI tract, and cardio-
pulmonary system).
Physiology of Swallowing
The swallowing process depends on a
highly complex and integrated sensorimo-
tor system. Swallowing is considered one
of the most complex functions because it
includes several anatomic areas, has vol-
untary and involuntary components, and
requires simultaneous inhibition of respi-
ration. Neuromuscular coordination must
engage the CNS, afferent sensory input,
motor responses of voluntary and invol-
untary muscles, the brain stem, and the
enteric nervous system (ENS). Hormonal
factors also play a critical role that is poorly
understood.
The integration of several normal func-
tions further complicates the act of swallow-
ing. These include chewing and swallowing,
respiration and chewing, and the pharyn-
geal phase of swallowing and respiration
(Miller, 1999). These functions, along with
the entire act of swallowing, are controlled
by pattern generators in the brain stem that

are modulated by the cerebral cortex as well
as through sensory input (Miller, 1999).
The historic interest in dysphagia has
provided a rich and detailed understand-
ing of the swallowing processes in adults,
especially those with neurologic deficits and
head/neck cancer. Although much of the
information may be applicable to the older
child, preterm infants, neonates, infants,
and young children have additional factors
of normal and abnormal development (see
later text) to consider. For the student inter-
ested in the neurophysiology of swallowing,
publications by Miller and coworkers are
recommended (Miller, 1999; Miller, Bieger,
Conklin, 1997).
Swallow Components/Phases
The swallowing process is commonly de-
scribed in phases or stages. Although the
functions needed to carry out the work
of each phase of swallowing may overlap,
for discussion purposes, swallowing is
described in five phases:
1. oral preparatory (also known as bolus
formation),
2. oral transit,
3. initiation of pharyngeal swallow,
4. pharyngeal, and
5. esophageal transit.
The first two phases are under volun-
tary neural control. The pharyngeal phase
has both voluntary and involuntary control.
The esophageal phase is under involuntary
control. The sequence of movements is dia-
grammed in Figure 2–10.
Oral Preparatory/Bolus Formation
The oral preparatory phase is voluntary and
requires a process for getting food and/or
liquid into the mouth. Someone needs to
feed the infant or child when age or neu-
rologic impairment precludes self-feeding.
Once food is in the mouth, formation of
a bolus begins. In a normal infant, bolus
formation per se is minimal. This phase
is characterized by latching to the nipple
(breast or bottle). Once liquid is extracted
from the nipple, the liquid is being trans-
ported posteriorly. When foods are added
to the diet, duration of the oral preparatory
phase varies considerably, depending on the
texture of the food and the child’s oral skill
level. As children begin to handle thicker,
lumpier textures, bolus formation may last
for several seconds. The more chewing that
is required, the longer it takes for bolus
preparation. Oral manipulation of liquid
presented via cup varies significantly from
one child to another, but usually liquid is
held in the oral cavity for less than 2 s.
Lip closure is needed once material is
in the mouth so that no liquid or food will
be dribbled down the chin. Some children
may move liquid (and at times food) around
in the mouth before they form a cohesive
bolus. The material is then held between
the elevated tongue and hard palate. The
digastric, genioglossus, geniohyoid, and
mylohyoid muscles aid in tongue eleva-
tion. The bolus is held in a median groove
in the tongue created by the movement of
the intrinsic muscles of the tongue, and the
lateral borders of the tongue abut the hard
palate (e.g., Derkay Schechter, 1998). The
buccinator muscles help to generate suction
in neonates and hold food between the teeth
in older infants and children. During this
process, the soft palate is in a lowered posi-
tion and resting against the tongue base.
This position helps to prevent a bolus from
entering the pharynx before the swallow is
produced. Active lowering of the soft pal-
ate occurs by contraction of the palatoglos-
sus muscle. The airway remains open and
nasal breathing continues until a pharyn-
geal swallow is initiated.

Oral Transit
Oral transit is under voluntary neural con-
trol and begins with posterior propulsion of
the food bolus by the tongue and ends with
the initiation of a pharyngeal swallow. The
voluntary actions in manipulating a bolus
of food or liquid include elevation and pos-
terior movement of the tongue, aided in
part by the styloglossus muscle. Sequential
contact of the tongue to the hard and soft
palate occurs as the bolus is propelled into
the pharynx. Elevation of the soft palate
against the posterior pharyngeal wall seals
the nasopharynx and prevents pharyngo-
nasal backflow, more commonly described
as nasopharyngeal reflux. Given the mate-
rial moves from the pharynx into the nasal
passage, it seems more accurate to use the
term pharyngonasal backflow (or reflux).
Oral transit timing does not vary according
to texture and is minimal in infants and less
than 1 s in children.
Initiation of Pharyngeal Swallow
The precise anatomic location for initiation
of the pharyngeal swallow is variable with no
published reports in children. Initiation may
occur at the anterior tonsillar pillars, base of
tongue, valleculae, or the pyriform sinuses
(Derkay Schechter, 1998). Asymptomatic
adults are seen to initiate pharyngeal swal-
lows with greater frequency in the valleculae,
whereas symptomatic adults and elderly per-
sons more often initiate pharyngeal swallows
in the hypopharynx and pyriform sinuses
(Zancan, Luchesi, Mituuti, Furkim, 2017).
Sensory input and feedback during
bolus formation and oral transit are criti-
Figure 2–10. Oral, pharyngeal, and esophageal components/phases of normal swallow in a
young child. A. Oral transit/phase showing formed bolus moving posteriorly through the oral
cavity. B. Initiation of pharyngeal phase. C. Bolus moving through the pharynx with adequate
airway protection. D. End of pharyngeal phase as upper esophageal sphincter (cricopharyn-
geus) opens. E. Esophageal transit with bolus in the cervical esophagus.

cal to normal swallowing. The rich and
diverse sensors include mechanoreceptors
(touch, pressure), pain receptors, proprio-
ceptive receptors (shape, location), chemi-
cal receptors, and special receptors for taste,
smell, and temperature. Interestingly, water
is perceived differently than other liquids,
particularly in the oropharynx (Miller,
1999). Mechanoreceptors located in the
tongue, teeth, soft palate, and hard palate
help to modulate the muscles of mastication
through brain-stem integrative pathways.
Pharyngeal Swallow Function
The pharyngeal swallow function is criti-
cal because the potential for aspiration is
greatest in this phase of the swallow. Sen-
sory input proceeds into specific regions of
the trigeminal nuclei (V) and the nucleus
tractus solitarius (NTS) of the brain stem
(Miller, 2008). Tongue base propulsion is
an important basis for pharyngeal swallow
initiation. The pharyngeal phase begins
with the voluntary production of a swallow
and the elevation of the soft palate to close
off the nasopharynx. Pharyngeal constric-
tors contract to propel the bolus through
the pharynx. Simultaneously, the larynx is
closed to protect the airway. There is no
interruption of the posterior bolus move-
ment with normal swallowing.
From a biomechanical perspective, the
pharyngeal swallow function can be divided
into six steps (Miller, 1999):
1. elevation and retraction of the soft
palate that results in closure of the
nasopharynx,
2. opening of the UES (relaxation and
passive opening with anterior laryn-
geal movement),
3. laryngeal closure at the level of the
laryngeal vestibule,
4. tongue loading or ramping,
5. tongue propulsion, and
6. pharyngeal clearance.
It was thought that the mylohyoid mus-
cle initiates this series of steps; however, the
genioglossus may be the first tongue mus-
cle to start the pharyngeal swallow (Miller,
1999).
Astheswallowoccurs,thelarynxengages
several mechanisms to provide protection:
1. Respiration ceases.
2. Laryngeal elevation and anterior
movement supported by the hyoid
bone bring the larynx under the base
of the tongue. Elevation contributes
to closure of the airway entrance
(minimal elevation occurs in young
infants given the larynx is high in the
neck). Forward movement contributes
to opening of the upper esophageal
sphincter (e.g., Logemann, 1998).
3. The epiglottis diverts food laterally
into the pyriform sinuses, although
not equally in all individuals.
Pyriform sinuses then open into the
esophageal inlet during simultaneous
cricopharyngeal (UES) opening.
4. Aryepiglottic folds move in an ante-
rior and medial direction to cover the
glottis.
5. Closure of the larynx (false vocal folds
and true vocal folds adduct) begins
at the level of the vocal folds and
progresses upward to the laryngeal
vestibule (Ardran Kemp, 1952,
1956).
The most important protection is the
complete and automatic closure of the lar-
ynx during swallowing. Vocal fold closure
occurs when the larynx elevates to approxi-
mately 50% of its maximum elevation (Gil-
bert et al., 1996). Contrary to popular belief,
the epiglottis is not absolutely essential for

glottic closure or for the prevention of aspi-
ration; however, it does play an important
and active role in most individuals. The epi-
glottis is brought down over the glottis dur-
ing swallowing and deflects the bolus being
swallowed material laterally and posteriorly
toward the esophagus.
High-speed cineradiography has been
used to distinguish two steps in closure of
the laryngeal vestibule (as opposed to the
glottis) during swallowing. The first step
observed was closure of the supraglottic
space of the laryngeal vestibule. Apposition
of the lateral walls seemed to be caused by
contraction and thickening of the supe-
rior portion of the thyroarytenoid muscles
(Ekberg, 1982). The second step was com-
pression of the subepiglottic space from
above as the posterior tongue movement
brought the epiglottis down over the laryn-
geal vestibule. This sequence of events sup-
ported the observation that a peristaltic-
like motion can clear the vestibule of bolus
material. Therefore, when the vestibule
is open after a swallow, it is free from any
residue of foreign particles.
Normally an infant swallows about
six times per minute while awake and six
times per hour while asleep. In infants a safe
swallow is aided by the cessation in breath-
ing and sustained laryngeal closure. This
mechanism is typically effective in protect-
ing the larynx from aspiration. During the
cessation of breathing, the swallowing rate
increases, presumably to clear secretions
from the airway before another breath is
drawn (Loughlin Lefton-Greif, 1994).
The increase in survival of preterm infants
has led to increased urgency for evidence-
based knowledge of the physical and physi-
ologic immaturity of these infants in order
to understand the difficulties many infants
have in feeding orally. This understanding
is needed in order to facilitate safe and effi-
cient oral feeding in these preterm infants
who swallow primarily during the cessation
of breathing and subsequent inhalation,
both of which increase the risk of oxygen
desaturation and laryngeal penetration/
aspiration (Lau, 2016; Lau, Smith, Schan-
ler, 2003).
Another major protective mechanism
for the airway is the cough reflex (Thach,
2007). Cough is triggered by sensory recep-
tors stimulated in the larynx and the sub-
glottic space and transmitted to the brain
stem by the vagus nerve (CN X). Immedi-
ately upon stimulation of these receptors,
the glottis is closed and an explosive cough
follows. Although limited data are available
regarding coughing and airway clearance
in infants and young children, mechanisms
associated with cough, provocation, and
resolution have been studied in premature
infants with bronchopulmonary dysplasia
(BPD). Coughing appears to have an upper
aerodigestive origin, while clearing appears
to be associated with peristaltic reflexes
(Jadcherla, Hasenstab, Shaker, Castile,
2015). Glottic closure reflex also aids in
protecting the larynx from noxious stimuli.
During swallowing, as the epiglottis
moves posteriorly and inferiorly, contrac-
tion of intrinsic laryngeal muscles brings
together the arytenoids, epiglottis, and the
false and true vocal folds. Simultaneously,
the larynx is elevated and pulled forward,
away from the path of the bolus. Laryngeal
function during swallow has been examined
in healthy young adults via frame-by-frame
analysis of concurrent transnasal video-
endoscopy, videofluoroscopy, pharyngeal
intraluminal manometry, and submental
surface electromyography (Shaker, Dodds,
Dantas, Hogan, Arndorfer, 1990). Four
sequential events associated with laryngeal
closure were noted: (a) adduction of the
true vocal folds associated with the horizon-
tal approximation of arytenoid cartilages,
(b) vertical approximation of the arytenoids

to the base of the epiglottis, (c) laryngeal
elevation, and (d) epiglottal descent. The
onset of vocal fold adduction was the first
event to occur in the oropharyngeal swal-
low sequence. Shaker and colleagues (1990)
noted that the mere introduction of liquid
into the mouth frequently caused the vocal
folds to adduct partially, suggesting that
there may be sensory afferent fibers within
the oral cavity that stimulate the laryngeal
closure protective mechanism. A simple
oroglottal reflex or higher brain-stem func-
tion may be involved.
Maximal vocal fold adduction preceded
the appearance of the peristaltic wave in the
oropharynx. The most striking finding by
Shaker et al. (1990) was that true vocal fold
closure was the first event to occur in the
oropharyngeal swallow sequence and that
it persisted throughout the sequence. The
vocal fold closure results primarily from
contraction of the intrinsic laryngeal adduc-
tor muscles, specifically the thyroarytenoids,
lateral cricoarytenoids, interarytenoids, and
cricothyroids. Previous studies (Barclay,
1930; Sasaki Isaacson, 1988; Sasaki
Masafumi, 1976) showed that the false vocal
folds closed during swallowing, but Shaker
et al. (1990) found that the false vocal folds
generally remained open. Infants and chil-
dren demonstrate cessation of breathing
with laryngeal closure that precedes poste-
rior bolus propulsion (Derkay Schechter,
1998; Loughlin Lefton-Greif, 1994).
Esophageal Swallow Function
Esophageal swallow function is character-
ized by an automatic peristaltic wave that
carries the bolus to the stomach. The pro-
cess of peristalsis moves the bolus through
the esophagus and ends when the food
passes through the gastroesophageal junc-
tion. The skeletal muscle in the cervical
esophagus propels the food more quickly
than the smooth muscle in the thoracic
esophagus. Primary peristalsis is triggered
in the pharyngeal phase of swallowing and
goes from the UES to the LES in one con-
traction. It is associated with cessation of
breathing during swallowing (Jadcherla,
2016). Secondary peristalsis is triggered by
esophageal provocation and is independent
of swallowing sequences. The waves occur
starting at the mid-esophagus and extend
to the stomach. These events participate in
propulsion of a bolus during swallowing
and also during gastroesophageal reflux.
The ENS, which was once dismissed as
a simple collection of relay ganglia, is now
recognized as a complex, integrative brain
in its own right that is capable of controlling
the GI function (e.g., Altaf Sood, 2008;
Kumral Zfass, 2018; Lake Heuckeroth,
2013). Its complexities are beyond the scope
of this chapter.
An esophageal phase promptly follows
each separate pharyngeal swallow when
there is a definite time delay between swal-
lows. As long as the bolus remains in the
striated segment, inhibition of the esopha-
geal phase occurs. When the bolus is in the
smooth muscle segment, delay in esopha-
geal transit of the initial bolus will occur. An
inactive, distended esophagus and continu-
ous LES relaxation may result from rapid
sequence swallowing seen during feed-
ing and increase the risk of gastroesopha-
geal reflux (GER). Some infants may have
esophageal propulsion after four or more
pharyngeal swallows. The esophageal peri-
stalsis may be delayed until the end of an
active burst of sucking. Swallow-induced
peristalsis normally propagates at about 2 to
4 cm/s and traverses the entire body of the
esophagus in 6 to 10 s in children (Arved-
son Lefton-Greif, 1998; Dodds, Hogan,
Reid, Stewart, 1973).
Solids have been shown to increase the
probability that a primary peristaltic wave

will progress through the entire esophagus
(Miller, 1982). LES function is dependent
on bolus size in adults with an increased
opening diameter and prolongation of the
interval of sphincter relaxation seen with
larger bolus volumes (Kahrilas, Dodds,
Dent, Logemann, Shaker, 1988).
Transient LES relaxations (TLESRs) are
brief periods of relaxation that are unre-
lated to swallowing or esophageal peristal-
sis. These transient pressure drops have
been attributed to relaxation of the smooth
muscle of the LES, although direct measure-
ments are difficult (Altaf Sood, 2008).
LES pressure is decreased by various
pharmacologic and hormonal influences.
Anticholinergics, theophylline, caffeine,
nicotine, alcohol, dopaminergics, epineph-
rine, and prostaglandins lower LES pres-
sure. GI hormones that lower LES pressure
include glucagon, secretin, cholecystokinin,
progesterone, and estrogen (Boeck, Buckley,
Schiff, 1997).
Mechanisms involved in normal acid
clearance include salivation, swallowing,
and peristalsis. All may be significantly
impaired in patients with swallowing disor-
ders. The sequence of events for acid clear-
ance is disrupted by drooling, decreased
numbers of swallows, and abnormal peri-
stalsis, all seen frequently in children with
oral sensorimotor dysfunction. Delay in
acid clearance sets the stage for a vicious
cycle of reflux esophagitis.
Normal function of the GI tract is nec-
essary for “normal” feeding in infants and
children. Esophageal motility and esopha-
geal and gastric competence are necessary
for a healthy upper digestive tract. Swal-
lowing and feeding problems are caused by
and contribute to the development of GI
disease in children. (See Chapter 5.) Less
obvious may be the role that proper intesti-
nal absorption and lower GI tract motility
play in the development of dysphagia. For
example, the cycle of dysphagia can result in
decreased fluid intake. Reduced fluid intake
leads to underhydration or dehydration.
The chronically low fluid intake, when com-
bined with relative immobility often seen in
children with neurologic impairment, can
lead to chronic constipation, resulting in
significant irritability during or after feed-
ings and in early satiety.
Prevention of excessive gastric contents
from returning to the esophagus and con-
tinuing upward beyond the esophagus, into
the pharynx, larynx, nose, and oral cavity,
is extremely important for the prevention
and maintenance of normal swallowing in
many infants and children. The physiology
of sphincters, mucosal protection, and the
role of swallowing in prevention of regurgita-
tion of gastric contents are described briefly.
Lower Esophageal
Sphincter (LES)
The lower esophageal (or gastroesopha-
geal) sphincter (LES) at the distal end of the
esophagus normally prevents free reflux of
gastric contents into the esophagus. A defi-
nite, anatomically defined sphincter, such
as that which exists at the pylorus, has not
yet been identified. However, a zone of
increased intraluminal pressure in the most
distal 1 to 3 cm of esophagus does exist.
During swallowing, a momentary relax-
ation of the LES allows swallowed food to
enter the stomach.
The LES muscle is an extension of the
esophageal circularmuscle of the body of the
esophagus. Although anatomically indistin-
guishable, the area of the LES muscle differs
from the circular muscle of the body of the
esophagus in that the LES demonstrates a
greater responsiveness to cholinergic stimu-
lation and more impressive length–tension
characteristics. Pressure generated by the
LES is important in maintaining sphinc-

ter competence. Several ligaments connect
the LES to the diaphragm and may aid in
maintaining sphincter function. The closed
lumen of the distal esophagus is collapsed
into an H-shape and is surrounded by a
collection of loose areolar tissue, providing
many of the attributes of a choke valve. The
angled entrance of the esophagus into the
stomach aids LES competence. This angle
produces function similar to that of a flap
valve. Intraluminal gastric pressure, aided
by the presence of gastric contents, may also
apply pressure on the esophageal lumen and
aid in sphincter competence.
The normal location of the LES is par-
tially in the abdomen. The pressure differ-
ential between the abdominal esophagus
(high pressure) and thoracic esophagus
(low pressure) helps to prevent the reflux
of gastric contents into the esophagus. The
stomach has a positive resting pressure of 6
to 10 mm Hg, and the thoracic esophagus
has a resting pressure of −6 to +10 mm Hg.
A pressure barrier of approximately 15 to 60
mm Hg must be generated to overcome the
LES and for stomach contents to reach the
esophagus. The important effects of abdom-
inal pressure on the LES are illustrated by
the existence of a hiatal hernia, which is a
rare occurrence in infants. A laparoscopic
approach to repair is feasible, even for neo-
nates (Petrosyan et al., 2018). A hiatal her-
nia exists when the abdominal esophagus
and part of the stomach rise up through the
diaphragm into the chest cavity. The LES is
then surrounded by negative intrathoracic
pressure. The intra-abdominal esophageal
pressure differential is gone, and the LES is
surrounded by a negative (instead of posi-
tive) pressure. Free reflux of gastric con-
tents into the esophagus occurs because of
absence of this pressure differential (Heine
Mittal, 1991; Sondheimer, 1988).
Several anatomic and physiologic mech-
anisms interact to contribute to the preven-
tion of reflux into the esophagus—sphincter
pressure, the mucosal choke mechanism, a
flap valve, intra-abdominal position, and
the anchoring by phrenoesophageal liga-
ments, especially by the right crus of the
diaphragm. The relative importance of each
of these mechanisms is not clear at this time.
However, it is believed that GERD/EERD is
prevented by several mechanisms relative to
the esophagus. At birth, the greater pressure
in the esophagus is the principal mechanism
of preventing reflux of stomach contents
(Boix-Ochoa Canals, 1976). In the first
few weeks after a term birth, the LES at the
gastroesophageal junction matures rapidly
and contributes to the prevention of reflux.
Esophageal bolus transport is recognized as
an equally important component of infant
oral feeding skills (Lau, 2016). Thereafter,
the pattern of esophageal swallow peristalsis
is essentially the same in infants, children,
and adults.
Airway and Gastrointestinal
Physiology
Airway Physiology
Proper oxygenation is essential for life and
necessary for safe oral feeding. Coordina-
tion and regulation of breathing and eating
matures during the first several weeks after
birth. During nutritive sucking in the first
week of life, normal preterm and full-term
infants often experience decreases in min-
ute ventilation, respiratory rate, and tidal
volume (Durand et al., 1981; Guilleminault
Coons, 1984; Mathew, Clark, Pronske,
Luna-Solazano, Peterson, 1985; Miller
DiFiore, 1995; Shivpuri, Martin, Carlo,
Fanaroff, 1983; Wilson, Thach, Brouillette,
Abu, 1981). Shortly after birth, these
physiologic aberrations disappear except

in children with neurologic compromise
(Rosen, Glaze, Frost, 1984).
Normally throughout oral feeds, infants
produce from one to three sucks before they
initiate a pharyngeal swallow. A short breath
hold may precede such a run. Although full-
term infants tolerate breath cessation rea-
sonably well, preterm infants may not and
therefore experience hypoxia more readily,
especially if they have underlying lung dis-
ease such as BPD (Garg, Kurzner, Bautista,
Keens, 1988). Preterm infants may initi-
ate a swallow during the period of breathing
cessation and inhalation that increases the
risk of oxygen desaturation and laryngeal
penetration/aspiration (Amaizu, Shulman,
Schanler, Lau, 2008; Fucile, McFarland,
Gisel, Lau, 2012). Term infants typically
swallow at respiratory phases that minimize
risks of aspiration during respiratory pauses
or when inspiratory airflow is minimized
(e.g., during exhalation or at end of inspi-
ration or exhalation) (Lau, 2016; Nishino,
2013). As the infant matures, suck and swal-
low occur in a 1:1 ratio, and the infant takes
a breath after a burst of suck and swallow
sequences (e.g., 10 to 30 sucks and swal-
lows, then a breath). Mathew et al. (1985)
found decreased minute ventilation second-
ary to a slower respiratory rate in 19 healthy
term infants during continuous nutritive
sucking. The mechanism of the decreased
respiratory rate was thought to be from the
inhibitory effects of liquid in the pharynx.
Minute ventilation was found to decrease
during continuous sucking, with return to
baseline during rest (Shivpuri et al., 1983).
Infant maturation leads to a reduction in the
degree of hypoventilation.
A normal pharyngeal swallow requires
complete bolus transport through the phar-
ynx and UES. This action must occur while
the body ensures protection of the airway
from aspiration of the swallowed material.
Posterior transport through the pharynx is
achieved via coordinated posterior tongue
propulsion, tongue base retraction, effective
pharyngeal constriction, and UES opening
by inhibition of tonic contraction (Cook et
al., 1989; Dodds, 1989; Loughlin Lefton-
Greif, 1994; Shapiro Kelly, 1994). Safe
transport through the upper esophagus
is achieved through precise coordination
between bolus transport and anterior supe-
rior elevation and closure of the laryngeal
complex, which assist in airway protection
(Derkay Schechter, 1998; Loughlin
Lefton-Greif, 1994; Martin, Logemann,
Shaker, Dodds, 1994; Rogers, Arvedson,
Msall, Demerath, 1993; Shaker et al.,
1990). During swallowing, normal persons
occasionally show transient barium spill-
over into the laryngeal vestibule above the
level of the true vocal folds. Aspiration does
not occur when complete vocal fold closure
is maintained throughout the swallow. That
said, competent glottic closure does not
mean that aspiration will not occur with the
resumption of glottic opening for breathing.
In the young infant, the airway may
be compromised by neck flexion, intrinsic
hypotonia of pharyngeal muscles, posterior
displacement of the mandible, and hyoid
bone compression. Airway patency is criti-
cal to the infant and is helped by appropriate
midline neutral positioning and muscle tone.
Elevation of the entire larynx occurs by
shortening of the thyrohyoid and suprahy-
oid muscles. The arytenoids come together
by contraction of the thyroarytenoids.
The epiglottis closes off the vestibule by a
vertical-to-horizontal movement achieved
primarily by thyrohyoid shortening. These
multiple levels of sphincteric action are
capable of closing off the trachea completely
from the pharynx and may prevent food or
liquid from penetrating into the trachea
during swallowing. As the bolus moves

through the pharynx, it usually divides so
that approximately half moves through the
pyriform sinus at each side of the pharynx
(see Figure 2–3). These two portions of the
bolus rejoin just above the level of the open-
ing into the esophagus. In some instances, a
greater portion of the bolus is seen moving
through one side of the pharynx and is not
considered abnormal.
The cricopharyngeus is closed during
quiet respiration. During swallowing the
UES opens as anterior–superior motion of
the larynx occurs with contraction of the
genioglossus and other muscles of the lar-
ynx (Derkay Schechter, 1998; Shapiro
Kelly, 1994). The resting tonic contraction
of the cricopharyngeus is initially inhibited
by the swallowing center through CN X
parasympathetic fibers (Derkay Schech-
ter; Doty, 1968). The closed UES assures
that no air enters into the esophagus during
inspiration. At the initiation of a pharyngeal
swallow, inhibition of tonic contraction of
the cricopharyngeus muscle allows a bolus
of food or liquid to pass from the pharynx
into the esophagus. The UES then closes
immediately after the bolus passes through it.
Elevated UES pressure at rest is neces-
sary to protect the pharynx from reflux of
esophageal or gastric contents. The inner-
vation of the cricopharyngeus muscle is
not well understood (Sasaki, 2000). Neural
innervation occurs via recurrent laryngeal
nerve and the superior laryngeal nerve
(Prades et al., 2009). According to Schech-
ter (1990), parasympathetic innervation
enters the muscle via CN X, as the source
of both contraction and relaxation. Schech-
ter described relaxation beginning when the
larynx moves anteriorly and superiorly by
the genioglossus and suprahyoid muscles.
The bolus is then carried into the esophagus
by a series of contraction waves, a continua-
tion of the pharyngeal stripping action.
Gastrointestinal (GI)
Physiology
The normal pattern of gastric motility and
gastric emptying (GE) represents the end
result of a variety of complex interactions.
Stomach function is influenced by myen-
teric neural and hormonal factors. Food
volume, physical state (solid or liquid), and
specific food content all affect GE (Siegel
Lebenthal, 1981). For example, the stom-
ach empties breast milk faster than formula
milk (Cavell, 1979; Meyer, Foong, Thapar,
Kritas, Shah, 2015). Increased concen-
trations of carbohydrates and proteins slow
GE. This effect appears to be mediated by
osmoreceptors because GE is delayed when
higher concentrations of glucose are pres-
ent (Barker, Cochrans, Corbett, Hunt,
1974; Cooke Moulang, 1972). The abil-
ity of starches and most proteins to delay
emptying as effectively as isocaloric solu-
tions of glucose and amino acids implies
that starches and proteins are broken down
into component glucose and amino acids
before affecting GE. Selective perfusion of
the jejunum and duodenum with hyperos-
motic solutions has localized osmoreceptors
to the duodenum, because perfusion of the
duodenum slows GE while perfusion of
the jejunum does not (Meeroff, Go, Phil-
lips, 1975).
Body position affects the rate of GE and
therefore the amount of gastric residue.
Premature infants are found to have similar
lower levels of gastric residue in the right
lateral and prone positions and higher levels
of gastric residue in left lateral and supine
positions. Yayan and colleagues (2018)
found the gastric emptying rate to be high-
est in the right lateral position at 30, 60, and
180 min and in the prone position at 120
min (Yayan, Kucukoglu, Dag, Karsavuran
Boyraz, 2018).

Gastric emptying is responsive to fats.
Long-chain fatty acids have a greater retard-
ing effect on emptying than do medium-
chain fatty acids when equal molar con-
centrations are compared (Siegel, Krantz,
Lebenthal, 1985). The “gold standard”
for measuring gastric emptying is techne-
tium scintigraphy, which requires radiation
exposure. Development of the C-acetate
breath test (C-ABT) standardized in healthy
children for GE of liquids (Hauser et al.,
2016a) and the C-octanoic acid breath test
(C-OBT) for GE of solids (Hauser et al.,
2016b). These techniques are determined
to be reliable and well accepted by parents
and children.
The mechanisms by which small bowel
receptors control emptying is not estab-
lished. Both neural and hormonal mecha-
nisms are possible.
Experimental animal and human clini-
cal studies have both indicated that when a
small amount of acid is instilled in the distal
esophagus, nearly all of the acid material is
cleared following the initiation of a single
swallow (Helm, Dodds, Pelc, Palmer,
Teeter, 1984). The low pH is not returned
to normal until successive swallows occur,
when saliva is delivered to the distal esopha-
gus. Saliva clings to the esophageal mucosa
and has an important role in mucosal pro-
tection. Saliva diverted by oral suction can
prevent return to baseline esophageal pH.
Awake adults with GERD/EERD have the
same salivary volume and buffering capac-
ity as those without reflux. During sleep,
however, the mean resting salivary flow is
very low in those who have GERD/EERD.
Decreased swallowing frequency during
sleep may also be responsible for prolonga-
tion of acid clearance time.
Patients with oral sensorimotor disor-
ders may be particularly prone to the devel-
opment of esophagitis by the mechanisms
explained previously. Excessive drooling
limits the amount of saliva to the esopha-
gus. Decreased rates and effectiveness of
swallowing impair esophageal clearance
frequency. All of these situations may inter-
rupt normal acid clearance and predispose
to GERD/EERD. See Chapter 5.
Neural Control of
Swallowing
Neural control of swallowing has been stud-
ied with electromyography, through lesion
studies of CNS pathways and peripheral
nerves, by removal of specific muscles, and
by electrical stimulation (Miller, 1986). The
neural control of swallowing involves four
major components (Dodds, 1989; Dodds,
Stewart, Logemann, 1990):
n afferent sensory fibers contained in
cranial nerves,
n cerebral, midbrain, and cerebellar fibers
that synapse with the brain-stem swal-
lowing centers,
n the paired swallowing centers in the
brain stem, and
n efferent motor fibers contained in
cranial nerves (Figure 2–11).
Swallowing can be evoked by many dif-
ferent central pathways, even after removal
of the entire cortical and subcortical regions
above the brain stem. This indicates that
the cerebral cortex is not essential to the
pharyngeal and esophageal phases (Miller,
1972), although the cerebral cortex appears
to facilitate the oral phase and the initiation
of the pharyngeal phase. Nonetheless, lim-
ited information is available regarding the
cortical control of both swallowing and res-
piration (Martin Sessle, 1993).

The relevance of the cerebral cortex to
motor control may relate to dependence
on the cortical region for the learning of
motor responses. Bilateral movements
of the face and tongue and repetitive jaw
movements have been observed by stimu-
lation of the prefrontal cortex with micro-
electrodes (Kubota, 1976). Stimulation with
larger electrodes and more current over the
same regions evokes swallowing (Miller
Bowman, 1977; Sumi, 1970). Although
extensive research has been carried out
over many years with important findings,
the underlying neural mechanisms for
both normal and disordered swallowing
remain vague (Humbert German, 2013).
These researchers suggest that principles of
motor learning based on limb movements
be used as a model system to provide a
basis for deeper understanding of control
of oropharyngeal function. Sensory input
is stressed as necessary for accurate motor
control. Sensory information is processed
during planning, executing, and evaluating
an action. Concepts of sensory feedback
and predictions prior to confirmation seem
especially significant for oropharyngeal
swallowing (Humbert German, 2013).
These principles are applicable across the
age span. However, the process of obtain-
ing evidence in infants seems even more
complicated. Thus, researchers and clini-
cians are always encouraged to consider
the interrelationships of sensory and motor
Fasciculus Solitarius
Medulla
Pons
and
Medulla
Motor Nuclei
Cranial Nerves
V, VII, IX, X, XII
Supranuclear Descending Pathways
Cortical and Subcortical
Primary Afferents
Cranial Nerves
V, VII, IX, X
Primary Efferents
Cranial Nerves
V, VII, IX, X, XII
Nucleus Tractus Solitarius
and
Ventral Medial
Reticular Formation
“Central Pattern Generator”
Figure 2–11. Major peripheral and central nervous system pathways for deglu-
tition. Significant afferents that include cranial nerves and subcortical pathways
send input through fasciculus solitarius to the nucleus tractus solitarius (NTS) and
the ventral medial reticular formation (VMRF) (central pattern generator). Efferent
nerves synapse with motor nuclei primarily of cranial nerves V, VII, IX, X, and XII.

learning. The term sensorimotor is useful in
a generic sense. In neonates, consideration
must be given to CNS, ENS, as well as neu-
romuscular effects on maturational delays,
maldevelopment, maladaptation, and mal-
function involving multiple systems (Jad-
cherla, 2016).
Sensory (afferent) cranial nerve input to
the brain-stem swallowing centers is pro-
vided mainly by the glossopharyngeal (CN
IX) and vagus (CN X) nerves, with some
contribution of the maxillary branch of the
trigeminal (CN V2) and facial (CN VII—
chorda tympani) nerves (Table 2–3). Stim-
uli that induce swallowing vary from one
region to another. Taste stimulation alone
is a weak stimulus for swallowing (Dodds,
1989), although the degree of sweetness
of the fluid appears to be one type of sen-
sory input for infants (Burke, 1977). Light
touch is the most effective stimulus at the
faucial pillars, heavy touch in the posterior
pharynx, and water at the posterior larynx
(Shinghai Shimada, 1976; Storey, 1968).
The faucial pillars, pharynx, and posterior
larynx provide sensory stimuli needed
to elicit a swallow (Miller, 1986; Steele
Miller, 2010). Peripheral nerve stimulation
to the posterior tongue and the oropharyn-
geal region innervated by the pharyngeal
branches of the glossopharyngeal nerve
(CN IX) or by two branches of the vagus
nerve (CN X—superior laryngeal nerve
and the recurrent laryngeal nerve) evokes
swallowing. Sensory fibers of these two
cranial nerves proceed centrally and syn-
apse within the nucleus tractus solitarius
(NTS). Microelectrode recordings indi-
cate that stimulation of the sensory fibers
evokes potentials within the NTS and in an
adjacent region, the ventral medial reticu-
lar formation (VMRF) around the nucleus
ambiguus, which is a vagal motor nucleus
(Car Roman, 1970; Jean, 1972; Miller,
1972, 1982). A small lesion within the dor-
somedial region of the NTS prevents the
sequence of esophageal contractions (Jean,
1972), and led Miller (1986) to suggest that
the core central pathway, which controls the
peristaltic activity of the esophagus, par-
tially resides near or in the NTS.
The brain stem contains the interneu-
rons essential to the swallowing response.
Each side of the brain stem has its own
central pattern generator, which is a com-
plete neural circuit capable of generating
the pattern without peripheral feedback.
Miller (1982) described two animal studies
(Roman, 1966; Roman Tieffenbach, 1972)
establishing that once deglutition is elicited
by stimulation of an afferent pathway, the
motor sequence of peristalsis will proceed
Table 2–3. Sensory (Afferent) Cranial Nerve Input for Swallowing
Cranial Nerve Function
Trigeminal (V2) General sensation, anterior two-thirds of tongue, soft
palate, nasopharynx, mouth
Facial (VII)
(chorda tympani)
Taste, anterior two-thirds of tongue, touch sensation
to lips
Glossopharyngeal
(IX)
Taste and general sensation of posterior third tongue;
sensation to tonsils, palate, soft palate
Vagus (X)–SLN Pharynx, larynx, viscera, base of tongue
Note. SLN = superior laryngeal nerve.

in the pharynx and esophagus, even with
esophageal transection or deviation of a
bolus. In contrast, Miller suggested that
peripheral feedback modifies the central
pattern generator as noted by the decrease
in the number of peristaltic waves by devia-
tion of a bolus in the esophagus.
These central pattern generators or
swallowing centers (NTS, VMRF, and
interneurons in the medulla) use many of
the same cranial motor fibers and cervical
muscles that are needed for coughing, gag-
ging, and vomiting functions (McBride
Danner, 1987). These swallowing centers
are not discrete focal areas but consist of
ill-defined broad areas located lateral to the
midline and ventral to the caudal portion of
the fourth ventricle, incorporating the NTS
and the VMRF. Sensory cranial nerve input
to the swallowing centers provides taste and
sensory information from the tongue and
oral–pharyngeal mucosa, as well as pro-
prioceptive information from the muscula-
ture involved. The swallowing centers also
receive input from rostral brain-stem cen-
ters, cerebellum, basal ganglia, and higher
cortical centers (McBride Danner, 1987).
Thus, bolus size, taste, temperature, loca-
tion, and consistency have well-defined
receptors and are sensed at many levels of
the CNS.
The sequential semiautomatic discharge
of neurons to groups of muscles of the
oropharyngeal, laryngeal, and esophageal
regions is the most characteristic property
of swallowing (Doty Bosma, 1956). Motor
neurons leave the swallow center to synapse
in cranial nerve nuclei on the ipsilateral
side. The lower motor neurons to the mus-
cles for swallowing reside in cranial nerves
V, VII, IX, X, and XII and the ansa cervicalis
(C1–C3), which joins to run with the hypo-
glossal nerve (CN XII). The ansa cervicalis
innervates some of the muscles in the neck
responsible in part for laryngeal elevation.
The motor nerves and their respective mus-
cle innervation are shown in Table 2–4. The
swallowing centers ensure the accuracy of
bilateral motor activity and proper sequenc-
ing of the muscles involved in swallowing.
Prevention of competing muscle activities,
for example, speech and respiration, allows
completion of the complex motor act of
deglutition without interruption (Kennedy
Kent, 1985).
Two functionally distinct central pattern
generators appear to be present for pharyn-
geal and esophageal swallowing function,
with the relevant interneurons residing in
different regions of the medulla. Stimula-
tion of a peripheral nerve to evoke swallow-
ing elicits activity in muscles ipsilateral to
the input, except for the middle and inferior
pharyngeal constrictor muscles, which are
controlled by the contralateral brain stem
(e.g., Aida et al., 2015). Animal studies have
established that once swallowing is elicited
by electrical stimulation of an afferent path-
way or by volition, the motor sequence of
peristalsis will proceed (Miller, 1982; Miller,
Bieger, Conklin, 1997). The fact that peri-
stalsis occurs without peripheral feedback
from an accompanying bolus indicates that
the mammalian neural control of peristal-
sis is governed by a central pattern gen-
erator. Protection of the laryngeal opening
from aspiration appears to be carried out
more effectively with stimulation of those
receptive fields innervated by the superior
laryngeal nerve (SLN of CN X). The role
of peripheral feedback is not clearly under-
stood. Some authors suggest that there may
be both facilitative and inhibitory inputs
(Miller Sherrington, 1916; Sumi, 1970).
Miller (1982) suggested that peripheral
feedback modifies the dominant central
control of swallowing.
Repetition rate of swallowing is modi-
fied by both the type of bolus and the pres-
ence of material within the pharynx, larynx,

or esophagus. The intensity and duration
of individual muscle activity in pharyngeal
and esophageal sequences vary with the
consistency of a bolus and ease of passage
through the tract. The genioglossus and the
geniohyoid muscles demonstrate a longer
duration of discharge with a more dense
consistency bolus (Hrychshyn Basmajian,
1972). Topical anesthesia to the mucosal
regions of soft palate, faucial pillars, tonsils,
base of the tongue, and the pharynx causes
an increase in the time required to evoke
repeated swallows (Mansson Sandberg,
1975). Electromyographic (EMG) studies
have shown that esophageal muscle activity
is of longer duration and higher amplitude
with a bolus of water compared with a bolus
of saliva (Miller, 1986). As the bolus proceeds
through the esophagus, continuous sensory
feedback occurs. Thus, primary and second-
ary peristalsis can be modulated as swallow-
ing occurs. Furthermore, bolus movement
is affected by intrathoracic pressure associ-
ated with changes in respiration. Specifically,
inspiration enhances movement and the
positive pressure associated with expiration
slows movement (Schechter, 1990).
Swallowing may depend on a central
patterned program (central pattern gen-
erator) that is modulated or reinforced by
feedback from sensory input, but it is not
dependent on this sensory input. Sensory
feedback modification of oral and pharyn-
geal swallowing processes may occur as a
preprogrammed modification governed by
proprioreceptors in the tongue that sense
the bolus size before initiation of a swallow.
It is also possible that sensory feedback
modification might occur online during the
swallowing sequence.
The neural control mechanism for
esophageal peristalsis in smooth muscle dif-
fers significantly from that for esophageal
striated muscle. It is generally agreed that
Table 2–4. Anatomic Location and Motor (Efferent) Controls for
Normal Swallow
Anatomic Location Innervation
Oral cavity
Muscles of mastication Trigeminal(V3) mandibular branch
Lip sphincter and face muscles Facial (VII)
Tongue-intrinsic muscles Hyoglossal (XII)
Extrinsic muscles Ansa cervicalis (C1-C2)
Palatoglossus Vagus (X)
Pharynx
Stylopharyngeus Glossopharyngeal (IX)
Palate, pharynx, and larynx Vagus (X)
Tensor veli palatini Trigeminal (V3)
Hyoid and laryngeal movement X, IX, V3, VII, C1-C2
Esophagus Vagus (X)

peristalsis in esophageal striated muscle is
determined by a descending sequence of
efferent neural discharges, generated by
the central swallow program (Diamant
El-Sharkawy, 1977). Esophageal smooth
muscle appears to be innervated by at least
two types of nerves (Dodds, Dent, Hogan,
Arndorfer, 1981), although the precise con-
trol mechanisms are controversial. It is not
clear whether a neural control mechanism
of esophageal peristalsis occurs as an “on”
response elicited by cholinergic nerves, or
an “off” response mediated by nonadrener-
gic, noncholinergic nerves (Dodds, 1989).
Nerve fibers that innervate esophageal
smooth muscle originate in the dorsal motor
nucleus rather than in the nucleus ambiguus
and synapse in the esophageal intramural
neural plexus, known as Auerbach’s plexus
(Ingelfinger, 1958). General agreement
exists that peristalsis in the esophagus, as
well as in the pharynx, occurs as a rapid
wave of relaxation followed by a slower wave
contraction. The rapidly descending wave of
inhibition relaxes the pharynx, UES, esopha-
geal body, and LES in a sequence to allow
the structures to accommodate an oncoming
bolus advanced by the peristaltic contraction
wave. The extrinsic component of the ENS
consists of both parasympathetic and sym-
pathetic divisions. This component is capa-
ble of modulating motility as well as other
functions in the GI tract that are beyond the
scope of this chapter (Altaf Sood, 2008).
Gravity assists peristalsis in persons who are
in an upright position.
Taste and Smell
Clinical concerns related to feeding prob-
lems in infants and young children usually
revolve around the motor mechanisms and
failure to handle changes in physical char-
acteristics of food, but the sense of taste and
smell also have important roles in feeding.
The addition of sucrose to fluid (water or
formula) has been found to aid in eliciting
suck-and-swallow patterns in infants (Weif-
fenbach Thach, 1973) and to increase
intake over a period of several weeks (Desor,
Maller, Turner, 1973; Foman, Ziegler,
Nelson, Edwards, 1983). Newborns are
generally responsive to breast odors (Win-
berg Porter, 1998) possibly facilitated by
the high norepinephrine release and arousal
of the locus coeruleus at birth. Two-day-old
infants recognize their mother by the moth-
er’s axillary odor, likely from influence of
skin-to-skin contact (Marin, Rapisardi,
Tani, 2015).
Food flavor preferences are shown to
relate to sensitive periods during which
infants seem most likely to form flavor
preferences and aversions that may pro-
vide the foundation for lifelong food habits
(Beauchamp Mennella, 1998). Mennella
and colleagues (2004) reported that varia-
tion in formula flavor affected acceptance
by young infants. A hydrolysate formula
is tolerated on first exposure to infants
when introduced less than 4 months of age
(e.g., Mennella Beauchamp, 1996, 1998).
Older infants strongly reject those formu-
las. These researchers suggest that there is
a profound change at about 4 months of
age in perception of those formulas and
that early experience modifies later accep-
tance. Breastfeeding offers an advantage in
initial acceptance of a food if the mothers
eat the food regularly (Forestell Mennella,
2007). Infant facial expression, although
not a true objective measure, is a response
that indicates food acceptance (Forestell
Mennella, 2017) and can be modified with
changes in experiences over time. Accord-
ing to Forestell and Mennella (2017), infants
who are breastfed by mothers eating varied

flavors, especially vegetables, tend to accept
those foods more readily when they are
ready for transition feeding. The increasing
variety in taste and smell of foods offered to
infants may be one of the prime factors in
the success of transitional feeding (Bosma,
1986). The interactions of chemosensory
cues and physical characteristics of food
continue to be studied. This is an area of
research that may aid in increased effec-
tiveness of intervention with some types of
feeding disorders.
Reflexes Related to Swallowing
A number of reflexes relate to swallowing.
Table 2–5 describes these reflexes in term
infants, their stimuli, the cranial nerves
involved, and the age of disappearance.
Some of these reflexes are more directly
related to the act of swallowing than oth-
ers. They include the gag reflex, phasic bite
reflex, transverse tongue response, tongue
protrusion, and rooting response.
The gag reflex consists of tongue pro-
trusion, head and jaw protrusion, and pha-
ryngeal contractions. A gag reflex is evident
by 26 to 27 weeks’ gestation and is usually
strong in full-term infants. A hyperactive
gag may be noted in some children with
neurologic impairment and often obvi-
ously a sensory response. Some children
gag at the sight or smell of food and others
when food is in the oral cavity prior to pos-
terior propulsion of a bolus. In some chil-
dren, a gag may be difficult to elicit when
profound motor dysfunction exists (Love
Webb, 1992). With ataxia, the gag may be
hypoactive. The absence of a gag reflex has
no relationship in and of itself to swallow-
ing. Children may have safe swallowing, but
no gag reflex. The gag reflex may diminish
somewhat at about 6 months of age, which
is usually marked by the onset of chewing
and swallowing of solids.
Phasic bite and tongue reflexes are pres-
ent by 28 weeks’ gestation. The phasic bite
reflex is the rhythmic closing and opening
of the jaws in response to stimulation of
the gums. Tongue protrusion is noted in
a full-term infant in response to touching
the anterior tongue. This tongue protru-
sion begins to diminish by 4 to 6 months of
age, permitting introduction of solids and
a spoon. The transverse tongue response is
Table 2–5. Infant Oral Reflexes Present at Term and Age They Disappear in Typical Infants
Reflexes Present
at Birth Stimulus Response
Cranial
Nerve
Age Reflexes
Disappear
Rooting Touch to cheek
or corner of the
mouth
Turns head toward
touch
V, VII,
XI, XII
3–6 months
Tongue protrusion Touch to tongue
or lips
Tongue protrudes XII 4–6 months
Tongue transverse Touch to tongue Lateral tongue
motion
XII 6–9 months
Phasic bite Pressure on gums Rhythmic closing V 9–12 months
Gag Touch posterior
tongue or pharynx
Contraction of
palate and pharynx
IX, X Persists

movement of the tongue toward the side of
stimulation when the lateral surface of the
tongue has been touched.
The rooting response, observed as the
head turns toward the side of stimulation
of the cheeks or the corner of the mouth, is
noted by 32 weeks’ gestation. It strengthens
gradually until term, when it becomes more
difficult to prevent an alert, hungry infant
from turning. Higher cortical pathways
cause inhibition by 3 to 6 months of age,
when the rooting reflex disappears.
In addition, several reflexes are initi-
ated in the fetus and newborn infant when
hypochloremic or strongly acidic solutions
(gastroesophageal reflux and particularly
laryngopharyngeal reflux that occurs mul-
tiple times a day in all infants) contact the
epithelium surrounding the entrance to
the laryngeal airway (Praud, 2010; Thach,
2001). These reflexes are known as the
laryngeal chemoreflex (LCR) and include
startle, rapid swallowing, apnea, laryngeal
constriction, hypertension, and bradycar-
dia. Praud (2010) stated that the role of
these upper airway reflexes is still debated
with uncertainties persisting regarding
treatment and prevention of potentially
dramatic consequences.
Development of
Feeding Skills
Suckling and Sucking
Underlying factors that are important to
facilitate oral feeding of preterm and term
infants at breast and with bottle/nipple in-
clude, but are not limited to, global neuro-
logic, airway, and gastrointestinal systems
(see other chapters for detailed informa-
tion about these topics). Suckling and
sucking are considered to be flexor skills.
Physiologic flexion (a characteristic of full-
term infants) is observed when the limbs
are flexed, whether the infant is in prone
or supine position. Some researchers dif-
ferentiate suckling and sucking, while oth-
ers use the term sucking for all. Thus, no
prescriptive guidance can be given for use
of terms, although differences that have
been described will be shared. Readers
have to make their own decisions until evi-
dence-based reports support a rationale for
terminology
During the 1st month after term birth,
infants maintain much of the physiologic
flexion as a result of the crowded space in
utero during the final weeks before birth.
This overall body flexion contributes to suc-
cessful oral feeding by allowing for attain-
ment of appropriate positioning relatively
easily. Two distinct patterns of the suck
occur in infant development, suckling and
sucking (Table 2–6 and see Figure 2–8).
Suckling, the first pattern to develop, is
acquired gradually in the second and third
trimesters and involves definite backward
and forward movements of the tongue
(Bosma, 1986; Morris Klein, 1987). Liq-
uid is drawn into the mouth through a
rhythmic licking (or stripping) type action
of the tongue, combined with pronounced
opening and closing of the jaw. Lips may be
loosely approximated and flared around a
nipple. The tongue moves forward for half
the suckle pattern, but the backward motion
is more pronounced. Tongue protrusion does
not extend beyond the border of the lips.
In contrast, sucking is the second pat-
tern that develops at about 6 months. The
body of the tongue raises and lowers with
strong activity of its intrinsic muscles while
the jaw makes a smaller vertical excursion.
Firmer approximation of the lips that must
be flared for efficient nipple-feeding along
with the pattern of tongue motion allows
for a negative pressure to build up in the

mouth. This combination of movements
works to get liquid and soft food into the
mouth. Strength of lip closure is a major fac-
tor in the shift of tongue patterns from pri-
marily an in–out to primarily an up–down
direction. The tongue has more room for
movement because of the downward and
forward growth of the oral cavity. The
action is sometimes referred to as “pump
sucking” because it resembles the action of
a pump handle (Morris Klein, 1987).
Similarities and differences noted
between suckle and suck patterns include
the following:
n Both patterns reveal a raising and
lowering of the jaw and tongue together
to create the pressure required to
express the liquid into the mouth.
n Sides of the tongue move upward to
form a central groove that helps in
formation of the liquid bolus and to
move the bolus posteriorly over the
tongue.
n The differences between suckling and
sucking are noted primarily in the
direction of tongue movement and in
the degree of valving or closure of the
lips (Morris Klein, 1987).
n The developmental sequence from
suckling to sucking is a step in prepara-
tion for oral manipulation of thick
liquids and advancement to spoon-
feeding of soft food.
The term sucking tends to be the generic
term and will be used to refer to the orga-
nized intake of a liquid or soft solid as
described in previous paragraphs. Suckle
will be used when emphasis is placed on a
specific developmental sequence of mouth
movements. Otherwise suck or sucking will
be used to describe typical infants younger
than 6 months of age and children with
developmental skill levels at those estimated
ranges in whom a mixture of suckle and
suck may be seen.
Patterns of sleeping and waking usually
determine the time intervals between feed-
ings during the first months of life. The pro-
cess of feeding at fairly regular intervals is a
major factor in establishing and maintain-
ing quiet arousal episodes or homeostasis.
Arousal helps prepare the infant for feed-
ing. Initially, arousal is noted when gross
motions of head, face, trunk, and extremi-
ties occur. Respiratory irregularities and an
increased respiratory rate are also noted.
Table 2–6. Suckling and Sucking Comparisons
Characteristic Suckling Sucking
Tongue configuration Flat, thin, cupped, or bowl
shaped
Flat, thin, slightly cupped, or
bowl shaped
Movement direction In–out movement horizontal Up–down movement vertical
Range of movement Extension or protrusion no
further out than middle of lip.
From mandible to the anterior
hard palate
Lip approximation Loose Firm
Expected ages/times Normal in early infancy Normal later infancy, childhood,
and adult

Incidental phonation is common before
crying. Crying may then become promi-
nent when there are delays in initiation of
the feeding process.
Nipple-Feeding:
Breast and Bottle
Breastfeeding
The normal suck–swallow sequence during
breastfeeding is similar to, but not the same
as other nipple-feeding options (McBride
Danner, 1987; Sakalidis Geddes, 2016).
Detailed guidance to facilitate breastfeeding
is beyond the scope of this chapter; however,
multiple resources are available for parents
and medical professionals (e.g., Casey,
Fucile, Dow, 2018). A brief discussion to
emphasize some basic factors follows.
Primary signs for a mother to make sure
her infant is well latched include the follow-
ing (Martin Zaichkin, 2016):
n a wide-open mouth with lips spread
(flared) around the breast,
n the infant’s mouth covering the entire
nipple and some of the areola,
n a firm tug on the breast with every suck
by the infant,
n the infant suckles for more than three
to four sucks in a row, and
n the infant maintains latch to the nipple
during pauses between bursts of sucks.
The tip of the tongue stays behind the
lower lip and over the lower gum, while
the rest of the tongue cups around the
areola of the breast. The mandible moves
the tongue up, allowing the breast areola to
be compressed against the infant’s alveolar
ridge. Milk is then expressed into the oral
cavity from the lactiferous ducts. While the
anterior portion of the tongue is raised, the
posterior tongue is depressed and retracted.
This forms a groove that channels the milk
to the posterior oral cavity where receptors
are stimulated to initiate a voluntary swal-
low. As the posterior tongue is depressed, the
buccal mucosa, supported by the buccinator
muscles and fat pads, moves inward slightly
and then outward while the mandible and
tongue are elevated during compression.
This movement of the buccal mucosa allows
for the maintenance of tongue approxima-
tion to the cheeks keeping milk within the
tongue’s groove (Smith, Erenberg, Nowak,
Franken, 1985). The jaw is then low-
ered, allowing the lactiferous ducts to refill,
and the sequence is repeated. A rhythm is
created by this sequence of vertical jaw
movement and posterior tongue depression
and elevation.
The suck–swallow sequence is repeated
approximately once per second as long as
milk is present and the infant is hungry.
The infant may interrupt feeding for rest
periods of various lengths. Feeders should
not interrupt a feeding as long as an infant
is coordinating sucking, swallowing, and
breathing sequencing (see Chapter 9 for
management guidance). Observe the infant
for cues for interruptions that may be part
of the total sequence (e.g., a few seconds
after every 8 to 12 suck–swallow sequences)
or at less predictable intervals. Although the
vertical jaw movement is a normal part of
the total sequence, excessive jaw excursion
may interfere with effective sucking. Some
infants use more of a biting action that is
painful for mothers and results in inad-
equate intake.
Most infants show a gradual decrease
in consistency of the rhythmic pattern of
sucking and swallowing with reduction in
force of the suck as the feeding progresses.
The duration of a breastfeeding session can

vary. After emptying each breast, infants
may continue to suck for pleasure.
Bottle-Feeding
In contrast, bottle-feeding infants can be
observed more directly regarding the vol-
ume consumed in a given time period.
Bubbles can be seen with every suck and
swallow in some “standard” bottles, but not
in some specialized bottles that have vent-
ing systems. Although each year brings new
bottles and nipples, there is no perfect sys-
tem. Individual differences are wide ranging.
Post-Feeding
A variety of behaviors may be noted in
the time immediately after feeding. Some
infants continue to hold the nipple in the
mouth and move it around with the tongue,
but without real sucking movements. Oth-
ers actively resist attempts to remove the
nipple by clamping the jaws together or
other types of struggle behavior. Newborn
infants tend to go to sleep directly after
feeding. Some infants give attention to the
environment, making this period an appro-
priate time for communication. Vocaliza-
tions can be imitated by the feeder, who can
also use this time for talking or singing to
the infant in a gentle voice, with the infant
held in a comfortable position allowing for
eye contact and touch in ways that yield
pleasurable interactions. These interactions
are an integral part of early communication
development, which is an ongoing process
beginning in utero.
Transition Feeding
In typical infants, the transitional feeding
period usually begins at 4 to 6 months of
age. The readiness for varied textures after
several months of suckle feeding is primar-
ily related to changes in the CNS, along
with some anatomic changes. Growth in the
upper aerodigestive tract occurs, but with
relatively minimal change in proportion or
form. There is an increase in intraoral space
as the mandible grows downward and for-
ward. The oral cavity also elongates in the
vertical dimension. The hyoid bone and
larynx shift downward resulting in altera-
tions to coordinate breathing and swal-
lowing. Breathing and swallowing truly
become reciprocal activities. The sucking
pads are gradually absorbed over the first
few months of life.
Eruption of teeth may be the most nota-
ble change in the peripheral anatomic struc-
tures. Mandibular teeth usually erupt before
the maxillary teeth, and girls’ teeth usually
erupt sooner than boys’ teeth (Moore, Per-
saud, Torchia, 2015). Deciduous teeth
erupt between 6 and 24 months after birth,
with all 20 deciduous teeth usually present
by the end of the 2nd year in most healthy
children. Mandibular incisors usually erupt
6 to 8 months after birth, but the process
may be as late as 12 to 13 months in some
normal children. Molars erupt from 12 to
24 months and the canines from 16 to 20
months. The erupted teeth are probably
more important as sensory receptors than
for motor purposes, because biting and
chewing during the transitional period can
be accomplished effectively with no teeth
on the “molar tables.” Biting and chew-
ing at this developmental stage are usu-
ally described as “munching.” The sensory
inputs of teeth may be significant in the
development of CNS control of the feeding
process (Bosma, 1986).
The resorption of the sucking pads,
eruption of molars, and enlargement of
the oral cavity all contribute to an increase
in the buccal space. As this buccal space
increases, food is manipulated between

the tongue and the buccal wall. The crush-
ing and grinding of food is assisted by the
molars (when present) or that portion of the
gums. Lateral tongue movements are basic
to manipulation of food in the oral cavity
as food is moved from midline to the lat-
eral buccal walls. It is common for infants
to approach their first spoon experiences
with suckling movements of the tongue.
The anteroposterior tongue movements
result in some food being pushed out of the
mouth. At times, these movements appear
similar to tongue thrusting. Gradually the
lateral tongue action becomes more consis-
tent with the rotary jaw action required for
efficient oral phase functioning.
The tongue continues to be a primary
contributor to normal oral feeding. Bosma
(1986) suggested that smooth food that is
homogeneous or with fine granular bits is
mashed by tongue gestures focused on the
midline of the tongue. As infants mature
they advance to semifirm food that requires
the tongue to move food to the lateral
buccal area, where it is mashed by verti-
cal motions of the tongue and jaw. These
manipulations appear to be a prelude to
chewing via molars. The motions of chew-
ing occur with or without erupted molars
in young children. Initial chewing gestures
are simple vertical mandibular movements.
Development of rotary jaw motion, jaw
motion speed, and management on consis-
tency upgrades are protracted during the
first 2 years of life in typical children (Wil-
son Green, 2009). As children continue
to develop, the vertical movements become
associated with alternating lateral motions
characteristic of mature mastication. Mas-
tication coordination is not observed by 30
months (Wilson Green, 2009). Mature
chewing is seen between 3 and 6 years of
age (Vitti Basmajian, 1975). Although the
precise developmental stages have not yet
been well delineated, normal infants and
young children demonstrate increasing
competence in the oral manipulation and
swallowing of varied food textures as they
get older.
As the ability to manipulate varied food
textures increases, parallel gains occur in
speech development as well as in trunk,
head, and neck stability (see the following
text). As the brain develops throughout the
first several months of life, sensory inputs
pertinent to feeding extend into the mid-
brain, cerebellum, thalamus, and cerebral
cortex. These developmental processes per-
mit the older infant and young child to gain
competence in the evaluation of the physical
character of food and ability to manipulate
and swallow it. Children who are in this
transition stage of feeding may still have
inconsistent suckle patterns, especially when
they are sleepy, distressed, or ill.
Termination of Nipple-Feeding
Many factors are considered when one thinks
about ending breast- or bottle-feeding. These
include age, culture, and a maternal desire
to maintain the bonding established with
breast- or bottle-feeding. By approximately
12 months of age, most children have several
teeth and appropriate CNS timing and coor-
dination capabilities to manage cup drinking.
Prolonged nipple-feeding has been identified
as a cause of dental caries, particularly when
sweetened liquid is taken immediately before
a sleeping period or intermittently during
sleeping periods. It appears that prolonged
bottle-feeding with sweetened formula and
juice has a greater effect as a cause of dental
caries compared with breastfeeding (Kotlow,
1977). Breastfeeding infants also can get den-
tal caries. Prolonged use of bottles, pacifiers,
and “sippy-cups” has been associated with an
increased incidence of otitis media (Niemela,
Pihakari, Pokka, Uhari, Uhari, 2000).

Normal/Typical Development
of Swallowing and Feeding
The evolution of feeding experiences is just
one aspect of a more generalized develop-
mentofthegrowingchild.Oralsensorimotor
skills improve within general neurodevelop-
ment, acquisition of muscle control (pos-
ture and tone), and psycho
social develop-
ment (e.g., Törölä et al., 2012). Cultural and
social factors within a family also influence
the feeding patterns. Culturally appropriate
techniques are important for monitoring
psychosocial development (Lansdown et al.,
1996), as well as expected feeding milestones.
Feeding is a complex developmental process
in which the infant or child and caregivers
all play active roles. The role of overall devel-
opment, including posture and muscle tone
and psycho
social development, is described
in the next section. The milestones described
reflect general concepts and should be con-
sidered within the specific cultural context of
the family. Clinicians must make decisions
with parents that incorporate family goals
within their respective cultures.
Normal Development
of Feeding Skills
Acquisition of age-appropriate feeding
skills is critical for the development of self-
regulation in infants and young children.
These early gains eventually lead to inde-
pendent oral feeding. The development of
socially acceptable feeding processes begins
at birth and progresses throughout the first
few years of childhood. Major strides in
sensorimotor integration of swallowing
and respiration, hand-eye coordination,
normal posture and tone development, and
appropriate psychosocial maturation are all
acquired during the critical first 3 years of
life (e.g., Delaney Arvedson, 2008). An
appropriate nurturing environment is fun-
damental to the emergence of high-quality
normal feeding and eating skills, to support
physical development, to acquire cognitive
and linguistic competence, and to secure
strong emotional attachments with care-
givers. These early skills lay the foundation
for normal physical growth and emotional
maturity extending through the adult years.
Normal developmental, position and pos-
ture, and psychosocial milestones for self-
feeding skills from birth to 36 months are
shown in Tables 2–7 and 2–8.
Neonatal and Early Infancy
Period (0 to 3 months)
Infant feeding behavior begins with a hunger
andsatietypatterninterspersedwithan irreg-
ular pattern of sleep and awake periods. Dur-
ing the first 2 to 3 months of life, a more regu-
lar pattern becomes established. The infant is
taking first steps toward self-regulation.
Coordination of breathing and eating
takes time to regulate, although postswallow
expiration is a robust feature of breathing–
swallowing coordination from birth (Kelly,
Huckabee, Jones, Frampton, 2007). Dur-
ing the first week of life, normal preterm and
full-term infants often experience decreases
in minute ventilation, respiratory rate, tidal
volume, and precise patterns of respiratory-
swallow coupling change (Durand et al.,
1981; Guilleminault Coons, 1984; Kelly
et al., 2007; Mathew et al., 1985; Shivpuri et
al., 1983; Wilson et al., 1981). Shortly after
birth, these physiologic alterations disap-
pear, except in children who are neuro-
logically compromised (Rosen et al., 1984).
Between 6 and 12 months, further matura-
tion of respiratory-swallow coupling occurs
most likely due to neural and anatomical
maturation (Kelly et al., 2007).

55
Table 2–7. Development/Posture and Feeding/Oral Sensorimotor Milestones,
Birth–36 Months
Age/Stage
Milestone
Development/Posture Feeding/Oral Sensorimotor
0–1 month
(Neonate)
Learning to control body against
gravity
Weight-bearing in prone (allows
head, neck, and shoulders freedom of
motion)
Head moves side to side in supine
position
Head lag when pulled to sit
Physiologic flexion
Posture to maintain pharyngeal airway
Strong grasp reflex
Suckle on nipple
Nasal respirations
Rooting reflex present
During feeding, hands fisted/
flexed across chest
Incomplete lip closure
Unable to release nipple
2 months
(Infancy,
2–6 months)
Emergence of improving tone and
symmetric purposeful movements
Improving head control
Exploring environment
Shifting weight toward chest and
moving arms forward in prone
Visual tracking
Sitting supported, head bobs
Preparing background movement for
future use (upper extremities coming
off surface to function in space;
weight shifting to pelvis, and lower
extremities move more freely)
Pelvis and lower extremities provide
additional support for upper extremities
Range of movement for jaw
Suckling pattern (anteroposterior
motion of tongue)
Mouth opens in anticipation of
food
Lip closure improved
Active lip movement with sucking
3 months Lifting head to 90° in prone
Lifting chest off the floor in prone
Weight-bearing on lower abdominal
muscles and pelvis in prone
Playing in space with flexion and
extension of neck in supine
Head participating in final half pull to
sit with fixation on examiner
Tolerating weight in supported standing
Early reflexes begin to fade
Nipple feeds continue
Neck flexion widens pharyngeal
airway
Midline orientation
Liquids
continues

56
Age/Stage
Milestone
4 months Gaining balance between flexor and
extensor development
Freeing arms for function in supine
and supported sitting
Controlling head in prone, sitting
Controlling head in mid-line through
pull to sit
Head in midline in supported sitting
Pivoting in prone
Rolling from prone accidentally
Rolling from supine actively
Playing with knees in supine
Tactile awareness in hands
Dissociating lip and tongue
Lip pursing
Blowing bubbles with saliva
Increased sound imitation
(cooing and laughing emerge)
Voluntary control of mouth
5 months Refining head and trunk control
Moving constantly
Rocking in prone
Opening hands
Playing with hands and feet in supine
Putting hands in mouth
Chin tuck to sit maneuver
Rolling actively from prone to supine
Holding nipple with center
portion of lips with balance and
stability
Tongue with small range of
up–down movement
Tongue reversal after spoon
removed, ejecting food
involuntarily
Sucking pattern emerging (uses
during spoon feeding)
Liquids, eating pureed
Gags on new textures
6 months Moving in a variety of directions
Pushing backward in prone
Reaching for toys; transfers from one
hand to another
Shows visual interest in small objects
Pulling up independently in pull to sit
Elongating of muscles increasing as
infant moves
Increasing upward movement against
gravity
Moving with a wide range of
up–down, forward–back tongue
and jaw movements
Pushing semisolid foods by
spoon out of mouth by tongue
Teething
Increased active oral exploration,
with toys, other objects, and
fingers
Rooting reflex, automatic bite
release are gone
Diminished gag reflex
Table 2–7. continued

57
Age/Stage
Milestone
6 months
continued
Displaying good spinal mobility and
rib cage expansion (necessary for
adequate respiratory coordination for
phonation and swallowing)
Longer lip closure
7–9 months
(late
infancy)
Crawling on belly, creeping on all fours
Full trunk control
Initiating movement from pelvis and
upper extremities
Changing position with lower
extremities as a base of support for
upper extremities
Moving smoothly
Development of extension, flexion,
and rotation has expanded what infant
can do in sitting
Pull to stand/hold on
Uses index finger to poke
Increased active head and neck to
lean forward
Gag reflex becomes protective
Mouth used for investigation of
the environment
Coordinated lip, tongue, and jaw
movements in all positions
Drooling only with teething
Cup drinking, lower lip as
stabilizer at 9 months
Mouth closure around cup rim
Moving lateral tongue to touch
solids while upper lip cleans off
spoon
Variegated babbling (mixture
of consonant and vowel
combinations, e.g., “ma,” “da”)
10–12
months
Full range of motion of upper
extremities
Changes position of lower extremities
independent of upper body
Stands independently
Learning to walk (cruising)
Pincer grasp (thumb and forefinger)
Smooth release for large objects
Self-finger-feeding
Increasing coordinated jaw,
tongue, and lip movements in all
positions
Weaning from nipple as cup
drinking increases
Easily closes lips on spoon and
uses lips to remove food from
spoon
Controlled sustained bite on cracker
Chews with up–down and
diagonal rotary movements
13–18
months
Walking alone
Using stairs
Grasp and release with precision
Scoops food to mouth
Movement in lips
Fully coordinated phonating,
swallowing, and breathing
All textures taken
Lateral tongue motion
Straw drinking
continues

Age/Stage
Milestone
19–24
months
Equilibrium improving Swallows with lip closure
Up–down tongue movements
precise
Self-feeding predominates
Chewable foods
Rotary chewing
Independent food intake
24–36
months
Refinement of skills of first 24 months
Jumps in place
Pedals tricycle
Uses scissors
Circulatory jaw rotations
Lip closure with chewing
One-handed cup holding and
open cup drinking without
spillage
Fills spoon with use of fingers
Solids
Total self-feeding; uses fork
The overall body posture of a normal
newborn is characterized by passive or phys-
iologic flexion. Trunk is neutrally aligned
and well supported for feeding, usually in a
semireclined position. Head and neck play
a primary role in feeding as they assume a
neutral to slightly flexed position with stable
support from the feeder (e.g., Wolf Glass,
1992). Head and neck posture/position is
an important factor in maintenance of the
patency of the pharyngeal airway (Bosma,
1988), with implications for the process of
craniocervical postural control. The rib cage
is positioned high and elevated in relation to
the trunk (Alexander, 1987). Postural con-
trol and normal sensorimotor development
involve the infant’s progression from primi-
tive mass patterns of movement to selective
movement against gravity (Caruso Sauer-
land, 1990) and the development of stability
and mobility.
The most important example of postural
stability in the newborn is the maintenance
of the pharyngeal airway. The muscles of the
pharynx adjust their contractions to main-
tain a constant diameter of the pharynx, so
gravity does not pull the tongue back into
the airway when an infant is in the supine
position. Similarly, the pharynx does not
collapse when the head is forward, although
a chin “tuck” is not advocated for young
infants. A chin tucked down toward the chest
may result in upper airway collapse. The
infant learns to control body against grav-
ity with the head moving side-to-side when
supine and weightbearing in prone to allow

59
Table 2–8. Psychosocial Milestones, Birth–36 Months
Stage Psychosocial Milestones
0–3 months
(homeostasis)
Cues for feeding: arousal, cry, rooting, sucking
Caregiver response leads to self-regulation
Quiets to voice
Hunger–satiety pattern develops
Interaction with primary caregiver becomes established with infant smile
Pleasurable feeding experiences lead to greater environmental interaction
3–6 months
(attachment)
“Falling in love”
Increased reciprocity of positive infant–caregiver interactions
Cues consistent
Anticipates feeding
Somatic functions stabilize
Pauses may be socialization (not necessarily satiety or for burping)
Laughing, smiling, alert, social
Parents are preferred feeders
Calls for attention (~6 months)
Means–end: repeats actions for toys, people, and things to evoke a
response (6 months)
6–36 months
(separation/
individuation)
Copies movements
Responds to “no”
Play activity to explore environment (7–9 mos.)
Uses facial expressions for likes and dislikes
Follows simple directions
Begins independent problem solving
Self-feeding emerging
Meal times become more predictable
Further experimentation with environment
Speech emerging
Speech very important
Follows two-step commands
Meals are increasingly linked to family schedule
Rapid increase in language
Independence complete

the head, neck, and shoulders increased
freedom of movement. Thus, a base of sta-
bility allows for increased mobility.
Neurodevelopmental milestones rele-
vant to normal feeding at this stage include
visual fixation and tracking and balanced
flexor and extensor tone of neck and trunk.
A variety of feeding positions may be used
for infant breast- and bottle-feeding. Care-
ful consideration must be given to the char-
acteristics of each infant. In general, infants
should be held in a fairly upright semiflexed
posture during feeding with head higher
than trunk for both breast- and bottle-
feeding. Infant–caregiver interaction during
feeds should begin to emerge with a smile
response by the infant at about 3 to 6 weeks
of age. The rooting reflex is present, along
with sucking and swallowing activities.
The psychosocial interactions during
feeding that occur between the infant and
caregiver (usually the mother) begin at
birth. The give-and-take exchange is neces-
sary for the emergence not only of adequate
feeding skills, but also positive behavior and
attitudes toward eating (Satter, 1999). The
normal newborn readily provides a set of
cues for the caregiver to recognize a need to
be fed (e.g., arousal, crying, rooting, suck-
ing). The infant should feed until satiety and
then demonstrate positive signs of fullness.
Responsive and attentive early feeding is
important in helping infants organize their
behavior and work toward the process of
self-regulation (Satter, 1990, 1995).
During the first 2 to 3 months of life, the
infant’s primary goal is to achieve homeo-
stasis with the environment. Sleep regula-
tion, regular eating schedules, and devel-
opmentally advantageous awake states are
some of the basic goals. Increasing interac-
tion with the environment allows the infant
to develop emotional attachment to the
primary caregiver(s) and others. Early feed-
ing skills can vary from one feeding to the
next and even across an individual feeding
(Thoyre, Shaker, Pridham, 2005). Infants
gain greater control of sucking, swallowing,
and breathing coordination for breast- and
bottle-/nipple-feeding. Reaching, smiling,
and social play are all fostered by pleasurable
and successful feeding experiences. Feeding
gradually becomes a social time (Greenspan
Lourie, 1981; Pridham, 1990; Pridham,
Martin, Sondel, Tluczek, 1989). Pauses
between sucking bursts become more
apparent and should not be interpreted as a
need for burping or early satiety. Although
uncommon, incorrectly interpreted breaks
in feeding can be associated with undernu-
trition (Whitten, Pettit, Fischoff, 1969).
If engagement between infant and caregiver
fails to develop, the infant may indicate lack
of pleasure, loss of appetite, and in its most
severe forms, vomiting and rumination.
Infancy (3 to 6 months)
Infants receive essentially all of their nour-
ishment through nipple-feedings for the
first 4 to 6 months. Breastfed and formula-
fed infants do not require additional types of
food through the 1st year. The World Health
Organization (WHO, 2001) and American
Academy of Pediatrics (AAP, 2012) recom-
mend exclusive breastfeeding for the first
6 months (Eidelman, 2012). AAP suggests
that infants be supplemented with oral iron
and vitamin D by 4 to 6 months until they
are eating age-appropriate iron-containing
foods (Iannelli, 2018, downloaded from
AAP website, 09/14/18). For healthy infants
at 4 to 6 months who are breastfeeding
exclusively, Smith and Becker (2016) in
a Cochrane review found no evidence of
benefit from additional foods nor any risks
related to morbidity or weight change. Thus,
they concluded that they could not disagree
with the recommendations by WHO and

AAP for exclusive breastfeeding for the
first 6 months of life in healthy infants. Most
typically developing infants begin taking
food when they reach the first transitional
feeding stage at 6 months. CNS maturation
allows the graduation from nipple-feeding
to transitional feeding with thin smooth
foods initially. Physical characteristics of
the face and mouth (particularly teeth) are
less important at this stage (Bosma, 1986).
However, not all parents follow that
6-month guideline. In Britain, 75% of Brit-
ish mothers introduced solids before 5
months, and 26% reported that decisions
were based on infants waking during the
night. A randomized clinical trial found that
early introduction of solids at 3 months was
associated with longer sleep duration, less
frequent waking at night, and a reduction
in reported serious sleep problems (Perkin
et al., 2018).
Self-weaning from breast or bottle to
a cup is related to the infant’s transition
toward self-regulation. A decreased inter-
est in sucking at the breast or from the
bottle often begins at about 5 to 6 months
of age. This coincides with developmental
advances and increased visual interest in
surroundings (Brazelton, 1969). Thus, by 4
to 6 months of age, considerations can be
made for spoon-feeding and, usually about
1 month later, the introduction of cup
drinking. Variability of commercially avail-
able cups is extensive in design parameters,
suction pressure, rate of flow, and residual
fluid with no one type that can be called
the “best” (Scarborough et al., 2010). Cau-
tion is urged with considerations based on
individual child characteristics. Cultural
variability needs to be considered. Success-
ful feeding requires appropriate reciprocal
relationships between caregivers and child.
Multiple factors need to be considered as
the child is learning to attain a sense of self
(Delaney Arvedson, 2008) involving a
balance between autonomy and depen-
dency that is often particularly revolved
around feeding.
Foods are introduced one at a time per
guidelines by dietitians as a means to prevent
or at least to minimize potential food aller-
gies (e.g., Fomon, 2001a, 2001b; Fiocchi,
Assa’ad, Bahna, 2006). Single ingredient
foods are recommended, which may vary
in different cultures. Gradually food with
texture can be added to make food thicker,
pastier, and grainier, but not chunky. Foods
that contain “pieces” in a thin liquid (e.g.,
vegetable soup) may result in coughing,
gagging, and at times vomiting. Introduc-
tion of those types of foods may precipi-
tate a feeding disorder as children become
scared and begin to refuse such foods that
may generalize to other foods over time.
A developmental “critical or sensitive
period” has been suggested for the intro-
duction of chewable textures in humans
(Illingworth Lister, 1964) and in other
animals (e.g., Denenberg, 1959). The term
critical period is applied to a fairly well-
delineated time period in which specific
stimuli must be applied to produce a par-
ticular developmental advancement. After
that critical time, the desired action can no
longer be learned. This can result in faulty
neurologic growth, resulting in long-lasting,
far-reaching negative impacts on multiple
systems. The term sensitive period is applied
to an optimal time for the application of
such stimuli, after which it is more difficult
and takes longer to learn a desired action or
pattern of behavior. For children with and
without developmental delays or disorders,
there is evidence that solid foods need to
be introduced at appropriate times or those
milestones of development will be missed.
If introduced at a later time, rejection of sol-
ids may then occur. The longer the delay,
the more difficult it is for many children to
accept texture changes.

By 6 months of age, foods (typically
smooth soft foods not requiring chew-
ing) on the tongue promote posterior pro-
pulsion followed by a swallow (Schechter,
1990). Interestingly, this skill development
coincides with the time when healthy chil-
dren begin to reach for objects. Children
who have motor coordination problems
due to cerebral palsy or other neuromotor
conditions may not yet be ready for these
foods. Illingworth and Lister (1964) posed
that withholding solids at a time when a
child should be able to chew (6 to 7 months
developmental level) can result in food
refusal and vomiting. As described later in
this chapter and in Chapter 13, psychosocial
development, personality, and environmen-
tal factors may complicate feeding problems.
The introduction of food to a child with
developmental delay can be challenging.
The time for introduction of solid foods is
estimated based on a developmental quo-
tient (DQ) that generally correlates to a level
of functioning. A comprehensive develop-
mental examination should yield an esti-
mated DQ that can be used to determine the
expected age of development for chewing.
The DQ can be estimated on the basis of a
variety of developmental scales (Chapter 3).
Most typical children with an average DQ
of 100 are ready for introduction of food at
usual ages. Variability may be observed in
the speed at which children move through
the steps of thin smooth to thick smooth to
slightly lumpy, to easily dissolvable and soft
chewable food.
Although there may be variability in
the speed at which children move through
the steps of thin smooth to thick smooth
to slightly lumpy, to easily dissolvable and
soft chewable food, the sequential order of
progression is relatively constant.
Developmental milestones during the
5th to 7th months include visual recogni-
tion of parents, followed by the recognition
of small objects, reaching, and grasping.
Parents are the preferred feeders, and the
child is now ready to assume an upright
posture during feeding.
Oral sensorimotor development is
supported by the overall development of
postural stability and associated increased
movements of the body. Postural control
develops within a range of muscle tension
that is not static and therefore allows for
adaptation to demands of the environment
(Langley, 1991). For example, by about age
4 to 6 months, increasing head control and
midline postural stability enable the tem-
poromandibular joint to control jaw open-
ing. By that age, the infant can open the
mouth wide in anticipation of a spoon or a
nipple. The jaw remains open in extension
until food has entered the mouth at which
time the jaw flexors take over. The develop-
ment of jaw flexor control occurs later than
jaw extensor control. Extensor and flexor
components gradually become balanced
so postural stability of the jaw appears con-
sistent by 24 months (Morris, 1985). The
increasing jaw stability permits increased
tongue and lip movements not only for
feeding, but also “sound” play. Vocalizations
occur in conjunction with oral movements.
For example, as the infant adapts to changes
in position or attempts to mold the body
into a caregiver’s arms, pleasurable cooing
or babbling sounds are likely to be produced
(Connor, Williamson, Siepp, 1978).
Late Infancy (6 months
to 1 year)
In the second 6 months of the first year,
four categories of feeding skills develop.
These skills include (a) taking food from
a spoon, (b) handling thicker and lumpier
foods that may require munching or chew-
ing, (c) self-feeding with fingers or a spoon,

and (d) drinking from a cup and manag-
ing the bottle independently (Pridham,
1990). These feeding skills emerge within
the broader context of oral sensorimo-
tor development, hand-to-mouth and fine
motor coordination, body positioning, and
communication.
Infants communicate interest in feeding
by their posture, head and mouth move-
ments, and vocalizations. As previously
noted, readiness for spoon-feeding usually
occurs around 4 to 6 months of age, when
a reduction in the typical anteroposterior
tongue action for suckling is seen. At age 5
to 7 months, the infant learns to get semi-
solid food from a spoon, and by about age
8 months, the infant can remove food from
the spoon quickly and efficiently (Prid-
ham, 1990). Healthy infants between 4 and
8 months of age were found to need an
average of 6 weeks (range of 2–10 weeks) to
acquire the skill of assisted spoon-feeding
(van den Engel-Hoek, van Hulst, van Ger-
ven, van Haaften, de Groot, 2014). A for-
ward head motion and use of both upper
and lower lips help bring the spoon into the
mouth. Improved trunk control and a stable
sitting posture enable improved head con-
trol (see text that follows). It is during the
second 6 months of the 1st year of life that
position and tone have the greatest impact
on the rapidly developing feeding skills.
The ability to sit without support is basic
to the ability to swallow thicker foods. At
about 6 months of age, oral-motor activity
is characterized by a kind of munching with
vertical movements of the jaw. At approxi-
mately 7 months of age, coincident with a
spurt in gross motor development, rotary
jaw action begins for chewing. Rotary chew-
ing is refined over the next 5 months. The
tongue’s increased flexibility, especially for
lateral motion, allows for a greater range of
bolus manipulation before swallowing. The
ability to manage a thicker bolus makes
feasible the introduction of soft food with
a lumpy texture.
Newtexturesshouldbeintroducedgrad-
ually. Mixed textures in the same bite tend
to be confusing to many children, particu-
larly to those with neurologic impairment.
For example, commercially prepared baby
food may contain chunky pieces mixed in a
liquid base. Some children find this difficult
to handle, and may appear unsure whether
to swallow it like liquid or to munch the
lumpier texture. The risks for choking and
aspiration are higher for a single bite of food
with a mixed texture rather than a homo-
geneous consistency. Illingworth and Lister
(1964) stressed that it is critical to introduce
lumpy textures at this stage of development
if the child is to learn to accept the consis-
tency. Otherwise, the probability increases
that the child will resist changes in texture
to a much greater degree than a child who
was introduced to lumpier textures within
the critical or sensitive time periods.
Chewing skills have been shown to vary
according to different textures (Saitoh et al.,
2007). In 143 healthy children, not surpris-
ingly, chewing time was found to be the
longest for solids and shortest for pureed
foods. The chewing time for viscous foods
was in between the time for the other two
textures (Gisel, 1991). Even though the
chewing time for solids was found to be lon-
ger than it was for other textures, children
developed mature chewing skills for solid
foods earlier than for viscous and pureed
foods. As expected, as children get older,
less chewing time is needed for all textures
(Gisel Patrick, 1988). The refinement of
rotary chewing patterns has been shown to
develop later, after 30 months. Rotary chew-
ing was not found in children studied at 30
months of age (Wilson Green, 2009). As
expected, as children get older, less chew-
ing time is needed for all textures (Gisel
Patrick, 1988),

Cup drinking is often introduced within
a month or two following the introduc-
tion of spoon-feeding. This developmental
advance often presents infants with new
challenges. Parents face challenges as well,
especially given the myriad types of “train-
ing” cups available in many areas of the
United States and other countries (Scarbor-
ough et al., 2010).
From 6 to 12 months, infants and their
families enjoy a burst of progress relative
to the development of posture and muscle
tone. As the infant gains trunk stability, the
extremities gain mobility, setting the stage
for self-feeding activities. In addition, with
neck and shoulder stability control, the
respiratory muscles, the larynx, and the
oral–pharyngeal structures gain stability.
The emergence of positional (external)
and postural (proximal and internal) sta-
bility is a prerequisite for the infant to be
able to reach for an object (Hadders-Algra,
2013). One arm may be held close to the
body to stabilize the shoulder girdle and
upper arm (by resting the elbow on the
chest), providing external stability. The fin-
gers can then open and reach for the object.
As time passes, internal or postural stabil-
ity emerges. The infant can now reach for
an object without needing external support
for the arm. Contraction of muscles around
the shoulder joint provides postural stabi-
lization necessary for movement of other
muscles. This postural stability enables the
child to perform distal movements more
freely and precisely.
With newfound motor skills, infants
begin to exert increased control over their
environment. Transition feeding, begin-
ning at age 6 months with spoon-feeding of
smooth purees, coincides with the begin-
ning of the developmental period of sep-
aration-individuation. (Chatoor, Schaefer,
Dickson, Egan, 1984). As young children
begin self-feeding, the mealtime experience
broadens from an intimate relationship with
a primary caregiver to participation in the
social event of the family meal. Similar situ-
ations occur in child care settings with staff
members and peers. Caregivers and chil-
dren typically work toward scheduled feed-
ing times that by the end of the first year
should coincide with family mealtimes.
Effective feeding includes selection of devel-
opmentally appropriate feeding methods, as
well as types and quantities of food. Older
infants need opportunities to achieve inde-
pendence in the feeding process.
It is during this period that self-control
must be balanced with independence. Fur-
ther discussion regarding roles of children
and their caregivers is found in Chapter 13.
As children become more independent in
eatinganddrinking,fewerfocusesareneeded
for oral feeding, that in turn allows intellec-
tual and social development to prevail.
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75
3Neurodevelopmental
Assessment of Swallowing
and Feeding
Brian Rogers and Shannon M.Theis
Summary
Successful swallowing and feeding repre-
sent the culmination of complex neurode-
velopmental processes within the frame-
work of each child’s physical well-being
and environment. The complex, integrated
neurologic and developmental processes
controlling or influencing swallowing and
feeding are represented in all levels of the
central and peripheral nervous systems (see
Chapter 2). Maturation and timing of these
processes are critical components of success-
ful swallowing and feeding as well as other
“streams of development,” including cogni-
tive, communicative, and motor skills.
Disorders of swallowing and feeding
in childhood are predominantly of neuro-
logic origin but are greatly influenced by
aerodigestive structure and function, gen-
eral health, and a variety of environmental
factors. The purpose of this chapter is to
provide an overview of important neuro-
developmental aspects of swallowing and
feeding. This overview will include a brief
discussion of central nervous system (CNS)
development and associated swallowing and
feeding skills, neurodevelopmental history
and examination methods, and case studies.
Morphogenesis of the
Human brain development is a prolonged
process that begins in the third gestational
week (GW) and extends through the life
span. Complex molecular events of gene
expression and environmental input inter-
act to guide brain development through the
traditional embryonic, fetal, and postnatal
periods. It is important to keep in mind
that these maturational processes are not
rigidly sequential in which one needs to be
completed before the next, but they overlap
and in many instances occur simultaneously
(Sarnat Flores-Sarnat, 2013). Disruption
of these processes can significantly alter
neurodevelopmental outcomes (Stiles
Jernigan, 2010). Emphasis will be placed on
normal and abnormal brain development
and its relationship to feeding, swallowing,
and general development.
Embryonic Period
The human embryonic period extends
through the 8th gestational week (GW). By
the end of this period, the major rudimentary

structures and compartments of the central
and peripheral nervous systems will be de-
fined (Sarnat, 2013; Stiles Jernigan, 2010).
By the end of the 3rd GW the embryo is
transformed through a series of processes
referred to as gastrulation, into a three-
layered structure (Stiles Jernigan, 2010).
The upper cell layer, composed of epiblasts,
will eventually give rise to all of the struc-
tures in the developing embryo. Among the
epiblasts, the neural stem cells or neuropro-
genitor cells appear between embryonic days
(E) 13 and 20 and are positioned along the
rostral-caudal midline of the upper layer of
the three-layer embryo resulting in the devel-
opment of the neural plate. These neuropro-
genitor cells will eventually give rise to all the
different cells of the brain and spinal cord.
Neurulation or the formation of the
neural tube occurs during the 3rd week of
gestation. The first sign of the neural tube
development is the appearance of two ridges
that form along the two sides of the neu-
ral plate at approximately E21. Over the
course of several days, the ridges rise and
fold inward to form the neural tube. Fusion
of the neural tube first occurs centrally and
then spreads rostral and caudal. When the
neural tube is closed, the neural progenitor
cells form a single layer of cells that lines the
center of the tube, adjacent to the hollow
center. Rostrally, the hollow neural tube will
give rise to the ventricular system, and the
adjacent neural progenitor cells will com-
pose the “ventricular zone” (VZ) that will
eventually form the brain. More caudally
located progenitor cells will give rise to the
hindbrain and eventually the spinal cord.
Between the 3rd and 8th weeks of the
embryonic period, there is a 10-fold increase
in the length of the embryo, and the shape
of the nervous system significantly changes.
The anterior end of the neural tube begins
to expand to form three primary brain ves-
icles, including the anterior prosencephalon
(forebrain), the middle or mesencephalon
(midbrain), and the posterior or rhomb-
encephalon (hindbrain). By the end of the
embryonic period, these three segments
further divide into the five secondary brain
vesicles. The prosencephalon divides into
the “telencephalon” and “diencephalon,” and
the rhomboencephalon divides into “met-
encephalon” (pons and cerebellum) and the
“myelencephalon” (medulla oblongata).
Disturbances of neurulation result in
various errors of neural tube closure rang-
ing from anencephaly (lack of forebrain
development with variable anomalies of
the upper brain stem that are incompatible
with life), encephalocele (restricted closure
of the anterior neural tube), and myelome-
ningocele (restricted closure of the poste-
rior neural tube). Myelomeningocele is usu-
ally associated with other brain anomalies
including Chiari type II malformation and
hydrocephalus. The Chiari type II malfor-
mation can result in various cranial nerve
deficits that may lead to significant swallow-
ing and feeding problems (see discussion on
cranial nerves).
Prosencephalic development refers to
the inductive influences of the prechordal
mesoderm that result in the formation of
the face and the forebrain. The peak period
of prosencephalic development occurs in
the 2nd and 3rd months of gestation (over-
lapping embryonic and fetal periods). Pros-
encephalic formation begins at the rostral
end of the neural tube at the end of the 1st
month of gestation. Prosencephalic cleav-
age occurs in the following 2 weeks and
results in the development of paired optic
vesicles, olfactory tracts, separation of the
telencephalon from the diencephalon, and
the sagittal cleavage of the telencephalon
to form the paired cerebral hemispheres,
lateral ventricles, and basal ganglia. Mid-
line prosencephalic development involves
the formation of three thickenings of tis-

3. Neurodevelopmental Assessment of Swallowing and Feeding 77
sue including the commissural, chiasmatic,
and hypothalamic plates. These structures
are important in the formation of the corpus
callosum, septum pellucidum, optic chiasm,
and hypothalamic structures, respectively.
Disorders of prosencephalic develop-
ment usually result in abnormalities of
both face and brain development. Abnor-
malities of prosencephalic formation that
are usually incompatible with life include
aprosencephaly (lack of development of
the telencephalon and diencephalon) and
atelencephaly (lack of telencephalon). The
holoprosencephalies result from failed
prosencephalic cleavages in the horizontal,
transverse, and sagittal planes. A common
anomaly includes the formation of a single-
sphered telencephalon and a less involved
diencephalon. Common disorders of mid-
line prosencephalic development include
agensis of the corpus callosum, septum pel-
lucidum, and septo-optic dysplasia. Com-
mon facial anomalies associated with these
disturbances of midline brain development
include hypotelorism, midline cleft lip and
palate, and a single front incisor. Children
with disorders of prosencephalic brain
development are at higher risk for vari-
ous developmental disabilities that include
intellectual disabilities, cerebral palsy (CP),
communication disorders, and neurogeneic
dysphagia.
Fetal Period
The fetal period of human development
extends from the 9th gestational week until
birth. During this time, the brain cortical
development goes through gradual but
striking changes from a smooth or “lissen-
cephalic” structure to the more recognizable
pattern of gyral and sulcal folding. These
gross anatomic changes reflect significant
changes at the cellular level, including cell
proliferation, neuronal migration, and post-
migrational cortical organization and con-
nectivity (Guerrini Dobyns, 2014; Stiles
Jernigan, 2010).
The human brain contains billions of
neurons. Most are produced by midgesta-
tion (Bayer et al., 1993; Rakic, 1995). Cell
proliferation begins in the embryonic period
(E42) and extends through midgestation in
most regions of the brain (Stiles Jernigan,
2010). The neural progenitor cells in the
ventricular zone undergo mitosis to form
neurons, which when formed are unable to
divide and form new cells. It takes only 33
mitotic cycles to produce all of the neurons
of the cerebral neocortex. Overproduction
of neurons in all parts of the neural tube by
30% to 50% is followed by apoptosis or pro-
gramed death of redundant neurons (Sarnat
Flores-Sarnat, 2013). Processes including
congenital infections or a genetic disorder
that arrests the proliferation of ventricular
zone neural progenitor cells sooner than the
requisite number of mitotic cycles are com-
pleted can result in micrencephaly or small
brain. Inadequate apoptosis of redundant
neurons has been speculated to be the basis
of macrocephaly or large brain in Sotos syn-
drome (Sarnat Flores-Sarnat, 2013).
The preplate plexus containing the
Cajal-Retzius or “pioneer” neurons of the
molecular zone are positioned to control
the expression of patterns of layer-specific
mRNA and protein expression, which
results in a laminar architecture of the neo-
cortical plate even prior to the first wave of
neuroblast migration (Hevner, 2007). This
laminar architecture and the resulting corti-
cal plate (cortex layers two through six) are
largely regulated by the gene Reelen (RELN)
contained on chromosome 7q22.1 in the
Cajal-Retzius neurons. Most of the migra-
tory neurons to the cerebellum and brain
stem arise from the margin of the primor-
dial fourth ventricle. At least six mutations

of the RELN gene have been found to cause
lissencephaly (smooth brain) with cer-
ebellar hypoplasia. Guerrini and Dobyns
(2014) identified 12 lissencephaly genes
that accounted for 90% of reported patients.
Lissencephaly is characterized by absent or
abnormally wide gyri plus an abnormally
thick cortex (Guerrini Dobyns, 2014).
Most patients with lissencephaly come to
medical attention in the first year of life due
to poor feeding, hypotonia, delayed motor
milestones, and/or seizures.
Neuroblast migration to the cerebral
cortex begins at 7 to 8 GW, and over 90% is
completed by 16 GW. In the cerebrum, spe-
cialized radial glial cells in the subventricu-
lar zone have long, slender processes along
which neurons migrate to the cortical plate
in waves, the earliest migrations forming
the deepest layers, and the last waves form
the most outer or Layer 2 cortical layer.
An additional tangential neuronal migra-
tion wave arises from the forebrain and is
responsible for GABAergic inhibitory inter-
neurons in the cortical plate that comprise
up to 20% of total cortical neurons (Sarnat
Flores-Sarnat, 2013).
Abnormalities of early neuronal migra-
tion have traditionally been classified based
on the effects on sulcation and gyration that
include lissencephaly, pachygyria, and poly-
microgryria. However, there often are abnor-
malities in the development of the brain stem
and cerebellum (Sarnat Flores-Sarnat,
2013). Disorders of early migratory arrest
include periventricular nodular heterotopia
and subcortical laminar heterotopia.
It is important to recognize that abnor-
malities of neuronal migration can result
not only from defects of genetic program-
ing, but also from acquired lesions during
the fetal period. Sarnat (1992) demon-
strated that severe telencephalic hypoplasia
or “smooth brain” could result from early
infarcts. Ischemic lesions have also been
linked to polymicrogyria. Congenital infec-
tions can cause vascular lesions that lead to
abnormalities of sulcation and gyration.
Axons form earlier than dendrites.
Axons sprout from migratory neurons
before they reach their destinations. Axo-
nal terminals proliferate to innervate many
neurons during maturation. In some brain
malformations, axons project to aberrant
sites of the brain. An example of a disorder
of axonal projection is agenesis of the corpus
callosum (Sarnat Flores-Sarnat, 2013).
The dendritic tree of each neuron starts
to proliferate only after the neuron reaches
its final site within the brain. Specialized
structures called dendritic spines form to
enlarge the synaptic surface and for spe-
cialization. There is a predictable timetable
for dendritic spine development in the neo-
cortex. Dendrites and axons form synapses,
and synapses allow the transmission of elec-
trochemical information that is the essential
means of communication between neurons
in the brain. Dendritic spine dysgenesis is the
underlying synaptopathology that is found in
many patients with intellectual and commu-
nicative disabilities, including Down, Rett,
and fragile-X syndromes and autism (Pen-
zes, Cahill, Jones, VanLeeuwen, Woolfrey,
2011; Phillips Pozzo-Miller, 2015).
Myelination
Myelination is the last stage of white matter
development that begins after axonal over-
production, pruning, and follows premye-
linating stages including the formation and
maturation of oligodendrocytes (Dubois
et al., 2014). This process includes the pro-
liferation and migration of oligodendrocyte
precursors to form “initiator” processes,
which align along axons and identify target-
ing axons followed by spiral ensheathment,
elongation, and wrapping around the axon.

This is followed by the myelin becoming
more compact. Myelin is a lipoprotein outer
cover for axons. Its function is to increase
the rate and efficiency of electrochemical
signaling down the axonal shaft.
Myelination is a marker of maturation
in the developing brain. Myelination of the
CNS begins as early as the 4th month of
gestation and continues in some regions of
the brain into the third and fourth decades
of life.
Areas of the CNS may differ in the
onset and rate of myelination (Kinney,
Brody, Kloman, Gilles, 1988; Yakovlev
Lecours, 1967). However, there are rec-
ognizable patterns of myelination of the
CNS. Its progression varies across cerebral
regions, following a caudo-rostral gradi-
ent and progressing from center to the
periphery. Proximal pathways myelinate
earlier and faster than distal pathways. Sen-
sory tracts myelinate before motor tracts.
Cerebral myelination occurs in projection
(e.g., thalamocortical) before associative
pathways (e.g., occipitotemporal path-
ways). Myelination, in general, progresses
from the central sulcus outward toward the
occipital, frontal, and temporal poles. Neu-
ral tracts mediating general proprioceptive
(position sense) and exteroceptive somatic
experience (tactile and pain), including the
medial lemniscus, outer division of the infe-
rior cerebellar peduncle, and the brachium
conjunctivum, myelinate beginning at
6 months’ gestation and extending to 1 year
of age. Myelination of specific thalamic
projection fibers to respective cortical areas
appears to be synchronized with cycles of
myelination of descending efferent cortico-
spinal and corticobulbar tracts from these
areas. Myelination of the corticospinal and
corticobulbar tracts appears initially near
term or 40 weeks’ gestation and increases
steadily with a “burst” at 8 to 9 months of
age. Myelination events are correlated with
motor-skill acquisition and other neurode-
velopmental milestones during the 1st year
of life. As myelination proceeds, the loss of
primitive or brain-stem-mediated reflexes
occurs. The Moro, asymmetric tonic neck,
and suckle reflexes are replaced by volun-
tary motor skills including rolling, sitting,
crawling, mature sucking, and vertical
chewing. The proximal to distal myelina-
tion pattern is manifested by the observed
motor pattern that batting or reaching for
objects appears before the development of
a voluntary grasp.
Myelination of the brain stem initially
appears at 5 months’ gestation. The myelin-
ation of the statoacoustic system (vestibular
and cochlear) commences at 5 months’ ges-
tation and is completed by 9 months’ gesta-
tion (term birth). At 5 to 6 months’ gestation,
the roots of cranial nerves III (oculomotor),
IV (trochlear), and VI (abducens), and the
intramedullary roots of cranial nerves VII
(facial), IX (glossopharyngeal), and XII
(hypoglossal) are myelinated.
A review of the neurophysiology of
swallowing is found in Chapter 2, but a few
key points are made concerning the synap-
togenesis and myelination of the nucleus
tractus solitarius and ventral medial reticu-
lar formation or central pattern generator
for swallowing in the medulla. The nucleus
solitarius (brain stem pneumotaxic center)
is synaptically mature before 15 weeks’ ges-
tation, coinciding with the appearance of
swallowing and onset of fetal respiratory
movements. Myelination of the tractus
solitariuis is a later event, commencing at
around 33 weeks’ gestation, and is not fully
complete even at term (Sarnat Flores-
Sarnat, 2016). Myelination of the reticular
formation around the nucleus ambiguus
and the nucleus tractus solitarius (site of
the central pattern generator for swallow-
ing) continues beyond 2 years of age. These
myelination patterns coincide with the

appearance of suckling at 18 to 24 weeks’
gestation and the continued development
and refinement of the oral and pharyngeal
phases of deglutition in the first few years of
life (see Chapter 2 for further discussion of
the neurophysiology of swallowing).
Hypoxia, metabolic disturbances, and
other complications occurring late in the
first and second trimester have been linked
to impaired rates of synapse formation in the
nucleus solitarius, and apnea of prematurity.
Symmetrical watershed tegmental infarcts
of the brain stem may involve the nucleus
solitarius, and account for the respiratory
insufficiency and dysphagia that may occur
in infants with Mobius syndrome (Igarashi,
Rose, Storgion,1997; Sarnat, 2004).
Prevalence of Swallowing
Information regarding the incidence/preva-
lence of swallowing and feeding disorders
in the general population of children and
various higher-risk groups has surprisingly
been somewhat limited. Better prevalence
data are gradually increasing in recent
years. Using the National Health Interview
Survey (NHIS) in 2012, Bhattacharyya
(2015) surveyed the general population
in the United States of children aged 3 to
17 years for voice or swallowing problems
lasting greater than 1 week. Out of the
total population of 61 million children, 569
thousand children (0.9% or nine per 1,000)
had a swallowing problem, but only 13%
were given a diagnosis for their swallow-
ing problem, and the most common cause
was “neurological problems.” Hvelplund,
Hansen, Koch, Andersson, and Skovgaard
(2016) surveyed all children born in Den-
mark from 1997 to 2010 (N = 918,280) for
the International Classification of Diseases,
10th Revision (ICD-10) diagnoses of feeding
and eating disorders (FEDs) in the first 48
months of life. They identified a cumulative
incidence of 1.6 per 1,000 live births. Pre-
term infants were more likely to have FEDs,
but over 84% of children with FEDs were
term infants. On univariate and multivari-
ate analyses, prematurity, small for gesta-
tional age, and congenital malformations
were strongly associated with FED. A sig-
nificantly increased risk of FED was seen
in girls, firstborn children, and children of
mothers who smoked during pregnancy.
A survey of all children between 4 and 7
years of age from a complete geographical
area in Germany was completed in 2008
by Equit and colleagues (2013). Parents
completed a 25-item questionnaire regard-
ing their child’s eating behavior as well as
anxious or oppositional behaviors. Interest-
ingly, 23% of the children were described as
only eating a narrow range of foods. Much
smaller percentages were noted to avoid all
foods (4.8%); have a profound refusal to eat,
drink, or be cared for (0.7%); or have a fear
of swallowing, choking, or vomiting (1%).
This survey as well as others have found
high rates of “picky” eating in the general
population of children.
A study in Thailand highlighted how
feeding problems affect feeding practices
in the home. Pediatricians interviewed the
parents of 402 children between 1 and 4
years of age (Benjasuwantep, Chaithiraya-
non, Eiamundomkan, 2013). The inves-
tigators found that 4.5% of the children
were described as having a limited appetite,
and 15% were described as having a highly
selective food intake. Children with feeding
problems were fed less frequently, were less
likely to be fed at their own table or at the
family table, and had mealtimes longer than
30 minutes.

Antecedents/Risk Factors
Birth Weight and Gestational Age
Very low (1500 grams), and particularly
extremely low (1000 grams) birth weight
preterm infants have consistently been
shown to be at much higher risk for feeding
problems compared to the general popula-
tion. In very low birth weight infants, the
prevalence of feeding problems has been
shown to decrease gradually from 25% in
the first year to about 6% at school entry
(Zehetgruber et al., 2014). Birth gestation,
duration of invasive ventilation, and the
presence of hypotonia at term age equiva-
lent have been identified as independent
predictors for feeding problems in very
low birth weight infants at 2 years of age
(Crapnell et al., 2013; Zehetgruber et al.,
2014). In a recent survey of all surviving
extremely low birth weight infants (born
at 25 weeks’ gestation or less) at 6 years of
age, eating problems were present in 35%
compared with 13% of age-matched con-
trols (Samara, Johnson, Lamberts, Mar-
low, Wolke, 2010). Oral motor difficul-
ties and “hypersensitivity problems” were
also much more common in extremely low
birth weight infants. Cognitive impairment
and neuromotor disability were associated
with increased prevalence of clinical oral
motor and hypersensitivity problems in this
cohort. Eating problems at 6 years in ex-
tremely low birth weight infants was sig-
nificantly correlated with poorer attained
growth, which was only partially explained
by other disabilities (Samara et al., 2010).
Infants with failure to thrive (FTT) or
undernutrition are particularly at risk for
both feeding problems (60%) and devel-
opmental delays (55%) (e.g., Raynor
Rudolf, 1996; Wright Birks, 2000). FTT
has recently been defined by the American
AcademyofPediatricsas“asignificantlypro-
longed cessation of appropriate weight gain
compared with recognized norms for age
and gender and may include weight-for-age
decreasing across two major centile chan-
nels from a previously established growth
pattern; and/or weight-for-length80% of
ideal weight, which is often accompanied by
normal height velocity” (Kleinman Greer,
2014). In a large, U.S. national population of
low birth weight (LBW) infants cared for at
university medical centers, the prevalence
of FTT was 19% in the first 3 years of life,
with a peak incidence rate between 4 and
8 months’ gestational corrected age. In this
cohort of LBW infants, factors associated
with FTT included small for gestation age,
abnormal neonatal neurodevelopmental
examinations, cognitive and motor delays
during infancy, and quality of home envi-
ronments (Kelleher et al., 1993).
Neurodevelopmental Factors
Association With Dysphagia
The strong association of dysphagia with
other neurodevelopmental disabilities has
advanced our understanding of the ante-
cedents of dysphagia in childhood. CP is
one of the major neurodevelopmental dis-
abilities, and it occurs in 2 of every 1,000
children. Dysphagia is common in children
with CP. A population-based study of 122
children in Australia with CP who were 18
to 36 months of age revealed a prevalence of
dysphagia in 85% of the cohort. All children
with greater degrees of motor impairment,
including those requiring assistance with
ambulation or postural instability, had sig-
nificant dysphagia (Benfer, Weir, Bell, Ware,
Davies, Boyd, 2013).
Gestational age, and particularly extreme
prematurity, has been strongly associated
with CP and dysphagia. The prevalence

of CP in term infants is one fortieth the
prevalence among extremely preterm sur-
vivors (Watson, Blair, Stanley, 2006). In
a recent study, McIntyre and colleagues
(McIntyre, Blair, Badawi, Keogh, Nelson,
2013) demonstrated that 91% of term and
near-term singletons with CP had no rec-
ognized asphyxiating birth event, but fetal
growth restriction and major birth defects
occurred substantially more frequently in
infants with CP than controls. Nelson and
Blair (2015) identified the predominance of
prenatal factors including gestational age,
birth defects, particularly brain and cardiac
anomalies, fetal growth restriction, prenatal
thrombotic states, placental pathology, and
genetics as causes of CP. Finally, CP has not
been shown to be preventable by a response
to electronic fetal monitoring (Nelson,
Dambrosia, Ting, Grether, 1996).
Although dysphagia in children is most
commonly associated with prenatal events,
there has been growing clarity regarding
what type of specific neonatal brain injuries
are most associated with serious swallow-
ing and feeding disorders. In term infants
with neonatal encephalopathy, watershed
brain injuries are most common, followed
by injuries to the basal ganglia and thala-
mus on cranial magnetic resonance imaging
(MRI) (Miller et al., 2005). Basal ganglia,
thalamic, and brain stem tegmental lesions
have been most closely linked to dysphagia
in young infants with suspected acute peri-
natal hypoxia-ischemia (Martinez-Biarge
et al., 2012; Quattrocchi et al., 2010).
Congenital Heart Disease and
In comparison to the general pediatric
population, infants and children with con-
genital heart disease are at higher risk for
swallowing and feeding problems. Over
half of infants with univentricular con-
genital heart disease (hypoplastic left heart
syndrome, single ventricle) require feeding
tube supplementation following their ini-
tial hospitalization. There are significant
increases in metabolic demands following
heart surgery that increase the risk for FTT.
Additionally, factors contributing to feeding
difficulty include vocal fold injury, uncoor-
dinated sucking and swallowing, genetic
influences, and growth hormone (Medoff-
Cooper Ravishankar, 2013). These fac-
tors as well as others result in higher rates
of growth failure in children with congenital
heart disease, which have been linked with
impaired executive function and worse
school performance (Bhoomika, Shobini,
Chandramouli, 2008; Black, Dubowitz,
Krishnakumar, Starr, 2007; Dykman,
Casey, Ackerman, McPherson, 2001).
Structural Anomalies and
Infants and children with specific structural
anomalies of the face, oral cavity, and neck
are at high risk for swallowing and feeding
abnormalities as well as neurodevelopmen-
tal delays or disabilities. At presentation,
the structural anomalies can often seem
to be the most obvious cause for feeding
problems, but the clinician should carefully
evaluate the presence of coexisting health
and neurodevelopmental disorders as either
contributors to or primary etiologies of the
swallowing and feeding problems. (See
Chapter 12.)
Tracheostomy and Association
With Dysphagia
Technology dependence may be associated
with developmental delays and feeding
problems. The prevalence of tracheosto-
mies in young infants cared for in neonatal
intensive care units has ranged between 1%

and 3% based on gestational age (DeMauro
et al., 2014; Overman et al., 2013). Broncho-
pulmonary dysplasia is the most common
reason for a tracheostomy in extremely pre-
mature infants, whereas structural airway
anomalies or congenital heart disease are
more common reasons for tracheostomies
in more mature high-risk infants (Overman
et al., 2013). (See Chapter 4.) A retrospective
analysis of the prevalence of dysphagia in 80
infants and toddlers with tracheostomies at
a regional children’s hospital revealed that
80% of the infants had dysphagia, with 81%
oral phase dysphagia, and 60% pharyngeal
phase dysphagia (Norman, Louw, Kritz-
inger, 2007). It is also important to note that
the majority of the infants were diagnosed
with gastroesophageal reflux.
Infants with tracheostomies, as a group,
are at significantly higher risk for neuro-
developmental delays/disabilities and dys-
phagia. In a multisite longitudinal follow-up
of 304 infants less than 30 weeks’ gestation
with tracheostomies, and 8,379 infants
without tracheostomies were evaluated
between 18 and 22 months of age by De-
Mauro and colleagues (2014). Infants with
tracheostomies were found to have cogni-
tive delays (77%), motor delays (68%), and
neurologic impairment (45%), while infants
without tracheostomies had significantly
lower rates of cognitive delays (30%), motor
delays (22%), and neurologic impairment
(7%) even after multiple adjustments of
factors predictive of adverse outcomes. The
presence of increased rates of neurodevel-
opmental impairments in infants with tra-
cheostomies was undoubtedly not the result
of tracheostomies, but due to the complex
reasons for the tracheostomies.
Abraham and Wolf (2000) investigated
the swallowing physiology of toddlers
with a history of long-term tracheostomy.
They found differences in the timing of
pharyngeal phase movements in patients
with tracheostomy versus no tracheostomy
during videofluoroscopic swallow studies
(VFSSs). In addition, delays in laryngeal
vestibule closure once anterior movement
of the arytenoids began was associated with
laryngeal penetration. In adults, placement
of one-way speaking valves has been shown
to improve swallowing function as well as
decrease laryngeal penetration and aspira-
tion with increased subglottic pressure dur-
ing swallowing (Dettelbach, Gross, Mahl-
mann, Eibling, 1995; Elpern, Borkgren
Okonek, Bacon, Gerstung, Skrzynski,
2000). Unfortunately, there is a dearth of
information regarding effects of swallow-
ing function and speaking valve placement.
In a pilot study of 12 consecutive pediat-
ric patients with tracheotomies who could
tolerate the Passy-Muir Speaking Valve
(PMSV) and with indications for a VFSS,
Ongkasuwan et al. (2014) found that place-
ment of the PMSV resulted in decreased
residue in the pyriform sinuses, but did
not demonstrate a statistically significant
effect in decreasing laryngeal penetration
or aspiration.
In Utero Constraint and
Children with various forms of in utero
constraint may be born with club feet or
multiple joint contractures (arthrogryposis
multiplex) and associated abnormalities of
limb movement, and may develop feeding
problems. Multiple joint contractures at
birth (arthrogryposis multiplex congenita)
occur with a frequency of 1:3,000 live births
(Hall, 1997). The primary cause of arthro-
gryposis is decreased fetal movements. Neu-
ropathic abnormalities including disorders
of the brain, spinal cord, peripheral nerves,
or muscles are the most common causes. In
a survey of 87 children with arthrogryposis
multiplex, 51 had major feeding problems

in infancy with the majority having difficul-
ties related to the tongue or jaw (e.g., chew-
ing or swallowing) (Robinson, 1990).
A variety of medications may influence
neurologic function, including motor devel-
opment, cognition, and feeding abilities.
A basic understanding of brain stem neu-
rotransmitter systems is helpful in under-
standing the impact of medications on swal-
lowing. Glutamate, excitatory amino acids
(NMDA [N-methyl-D-aspartate receptor]
agonists)andmonoamines(dopamine)stim-
ulate, and catecholamines (clonidine) have
been shown to inhibit swallowing in various
animal models (Jean, 2001). Table 3–1 lists
some medications and their actions on both
the central and peripheral nervous systems.
Commonly used anticonvulsants (including
carbamazepine, gabapentin, phenobarbital,
phenytoin, and valproic acid) and muscle
relaxants, including baclofen and cycloben-
zaprine, may produce drowsiness (Balzer,
2000). Benzodiazepines are used as anti-
convulsants and occasionally for treatment
of spasticity. In addition to their sedative
effects, benzodiazepines may directly reduce
activity in brain stem centers that regulate
swallowing (Buchholz, 1995; Wyllie, Wyllie,
Cruse, Rothner, Ehrenberg, 1986). Dopa-
mine antagonists, including the neurolep-
tics, are often used for agitation and aggres-
sive behavior in children with cognitive and
communicative impairments. These medi-
cations have been associated with the devel-
opment of laryngeopharyngeal dystonia
and esophageal dysmotility (Moss Green,
1982; Sokoloff Pavlakovic, 1997; Sico
Patwa, 2011). Selective serotonin reuptake
Table 3–1. Medication-Induced Nervous System Abnormalities Related to Dysphagia
Nervous System Abnormalities Medications
Central nervous system
Arousal Benzodiazepines
Chloral hydrate
Hydroxyzine
Antihistamines
Neuroleptics
Anticonvulsants (barbiturates, valproate,
carbamazepine, gabapentin, phenytoin)
Suppression of brain stem regulation Benzodiazepines
Movement disorders (e.g., tardive dyskinesia) Dopamine antagonists (e.g., neuroleptics)
Muscle relaxation Baclofen
Peripheral nervous system
Neuromuscular junction blockade Aminoglycosides
Myopathy Corticosteroids
Diminished salivation Anticholinergics (e.g., tricyclic
antidepressants and antihistamines)
Source: Adapted from Arvedson, J. C., Rogers, B. T. (Eds.). (1997). Pediatric dysphagia: Management
challenges for school-based speech language pathologist. Pittsburgh, PA: Rehabilitation Training Network
Health Care Group. Copyright 1997.

inhibitors (SSRIs) and tricyclic antidepres-
sants can cause xerostomia or dry mouth,
which occasionally may impact swallowing
(Balzer, 2000). There have been a number
of case reports of worsening dysphagia in
children with CP associated with botulinum
neurotoxin type A treatment for spasticity
(Montastruc et al., 2017).
In summary, the prevalence of serious
swallowing and feeding problems ranges
from 1 to 10 per 1,000 children, and these
problems are generally more common dur-
ing the first 2 years of life. Most children
with feeding problems are term infants.
Those at particular high risk for feeding/
swallowing problems are preterm or infants
who are small for gestational age and those
with congenital malformations. Infants and
children at high risk for dysphagia include
those who present with FTT, hypotonia,
neurodevelopmental disabilities including
CP, and/or have a history of hypoxic isch-
emic brain injuries, particularly those with
associated basal ganglia/thalamic or brain
stem tegmentum injury.
Clinical Evaluation
Neurodevelopmental
History (Basis for First
Step in Evaluation)
The CNS is the primary determinant for
Gesell’s maturational model of general
development and feeding (Gesell, 1940).
Swallowing and feeding are best viewed as
complex neurodevelopmental skills with
very close linkages to general health and
environment factors. In general, dysphagia
and neurodevelopmental delays or disabili-
ties typically coexist in children.
Abnormalities of the developing brain
commonly result in a spectrum of cogni-
tive, communicative, behavioral, and motor
abnormalities that are often associated with
swallowing and feeding disorders. Develop-
mental disabilities and swallowing and feed-
ing disorders share many important risk
factors and are influenced by similar health
conditions. An accurate developmental
history is dependent on the appreciation of
both the strengths and potential weaknesses
of caregiver’s reports. Finally, the concepts
of developmental delay, dissociation, devi-
ancy, and their usefulness in developmental
diagnosis will be reviewed.
The development of children is a pro-
cess that optimally should be assessed over
time. Arnold Gesell’s description of the
maturation of the four major “streams”
of development—communication, visual
problem-solving, motor, and social/adap-
tive skills—helped establish the field of
infant development (Gesell, 1940). Esti-
mates of the quality and rate of the four
major streams of development should be
made over time, avoiding a “cross-sectional”
approach to developmental assessment.
Feeding is also a developmental skill and
should be assessed longitudinally.
The neurodevelopmental history begins
with professionals asking parents about
their perceptions of their child’s develop-
ment and if they have any questions or
concerns. These subjective questions have
been found to be reasonably useful to pro-
fessionals in screening general popula-
tions of children for various developmental
delays. Certain parental concerns regard-
ing motor, language, and global/cognitive
skills have been found to have high levels
of sensitivity and have identified up to 80%
of children with disabilities (Glascoe, 1997,
2000). Nonetheless, the lack of parental
concerns does not reliably identify infants
who are developing normally in the general
population or in high-risk preterm infants
(Glascoe, 1997; Rogers et al., 1992). This

inaccuracy was confirmed in a recent study
utilizing the Parents’ Evaluation of Devel-
opmental Status (PEDS) (Limbos Joyce,
2011) and the Ages and Stages Question-
naires (ASQ) (Squires, Potter, Bricker,
1999) in a general pediatric practice that
demonstrated moderate sensitivity but low
specificity for the PEDS.
Parent-reported current developmental
milestones have proven to be more accurate
developmental screening measures com-
pared with parental concerns. Instruments
including the ASQ, The Child Development
Inventory (CDI) (Ireton Glascoe, 1995),
the Clinical Linguistic and Auditory Mile-
stone Scale (CLAMS) (Capute et al., 1986),
and the Motor Quotient (Capute Shap-
iro, 1985) have proven to be useful develop-
mental assessment measures. Most of these
assessments have defined developmental
delays through the use of standard devia-
tions or developmental quotients. Capute
advocated the use of developmental quo-
tients (Capute Shapiro, 1985). Develop-
mental quotients can be calculated for all
four of the major streams of development.
Accurate normalized population data with
generated means and standard deviations
for various developmental milestones are
required for the construction of develop-
mental quotients. A developmental quotient
is defined as the ratio of the development
age of the child divided by the chronologic
age multiplied by 100. Table 3–2 contains
normalized data for common motor mile-
stones in the first 2 years of life (Capute
Accardo, 1996; Capute, Shapiro, Palmer,
1985). The motor quotient of a 24-month-
old, full-term infant who has just started to
walk independently is the motor age (12
months) divided by the chronologic age
(24 months) multiplied by 100, which is 50.
Motor quotients used between the ages of
8 and 18 months have been shown to have
high degrees of sensitivity and specificity in
identifying infants with continued motor
delays at 24 months of age, including those
with CP (Capute Shapiro 1985).
TheWorldHealthOrganization’s(WHO)
internationalmulticenterlongitudinalgrowth
reference study of 816 children in the first
2 years of life included the documentation
of six motor milestones. The motor mile-
stones and the 50% for age of attainment of
this cohort included sitting without support
Table 3–2. Normalized Data for Common
Gross Motor Milestones in the First
15 Months of Life
Gross Motor Milestones
Age
(months)
Head up in prone position 1
Chest up in prone position 2
Head control 3
Up on hands or wrists in
prone position
4
Roll over (Prone ➝ Supine) 4
Roll over (Supine ➝ Prone) 5
Sit (anterior propping) 5
Sit (lateral propping) 7
Creep (belly crawl) 7
Crawl (on hands and knees) 8
Come to sit 8
Pull to stand 8
Sit (posterior propping) 9
Cruise (walk along furniture) 10
Walk (independently) 12
Walk backward 14
Run 15
Source: Adapted from Capute, A. J., Accardo, P. J.
(1996).The infant neurodevelopmental assessment:
A clinical interpretive manual for CAT-CLAMS in the
first two years of life, part 2. Current Problems in

at 5.9 months, standing with assistance at
7.4 months, hands and knees crawling
at 8.3 months, walking with assistance at
9 months, standing alone at 10.8 months,
and walking independently at 12 months
(WHO Multicentre Growth Reference Study
Group, 2006). These milestones are quite
similar to Capute’s data. It is extremely help-
ful to have a very good appreciation of the
close association between development of
oral motor and self-feeding skills and fine,
gross motor, and cognitive development in
children. A key sequence at about 3 months
of age is the development of stabilized trunk
and neck control and midline orientation of
head and hands. Specifically, normative data
indicate that infants hold their heads in line
with their trunks while being pulled to a sit-
ting position from supine, and keep their
heads steady and in line with their trunks
while being held in a supported sitting posi-
tion on their caregiver’s lap (Sheldrick
Perrin, 2013). At the same time, infants can
be observed to hold their hands together in
midline and their heads in midline while
in a supine position. On clinical examina-
tion at 3 months of age, young infants who
are lying in a supine position will look the
examiner straight in the eyes while keeping
their heads and hands midline. Coinciding
with the development of independent sit-
ting, most infants by 7 months will dem-
onstrate cognitively and motor-driven pre-
hension milestones, including reaching and
grasping a 1-inch cube, picking up a cup by
5 months, and securing a small pellet from
the table surface by 7 months (Accardo
Capute, 2005; Carruth, Ziegler, Gordon,
Hendricks, 2004; Sheldrick Perrin, 2013).
These skills set the “stage” for the appear-
ance of efficient finger feeding by over 96%
of infants by 7 to 8 months of age (Carruth
et al., 2004).
Cause-effect play appears in the latter
half of the first year and can be demon-
strated by observing infants purposely ring-
ing a bell by 9 months (Accardo Capute,
2005). This cognitive milestone coincides
with the appearance of infants eating foods
that require purposeful chewing (Carruth
et al., 2004). A number of infant develop-
mental scales highlight the appearance of
infants using objects for a purpose, such
as using a stick to obtain a toy by 19 to 20
months (Accardo Capute, 2005). This
cognitive skill is manifested by the appear-
ance of using a spoon without much spill-
age, and drinking from a regular cup by 19
to 20 months (Carruth et al., 2004).
The concept of developmental disso-
ciation involves comparison of the rates
of the four major streams of development.
The appreciation of the pattern of delays of
these streams of development is essential
in the diagnosis of neurodevelopmental
disorders associated with swallowing and
feeding abnormalities. Table 3–3 includes
examples of the presentations of children
with major neurodevelopmental disabili-
ties that include intellectual disabilities,
communication disorders, and CP. Chil-
dren with intellectual disabilities generally
present with significant delays in communi-
cation, visual problem-solving, and social-
adaptive skills. In contrast, children with
communication disorders have significant
delays in receptive and expressive language
and possibly social-adaptive skills, but gen-
erally normal visual problem-solving skills.
Clinicians need to assess both the
sequence and the quality of developmental
milestones provided by parents or caregiv-
ers. All streams of development are gener-
ally sequential and orderly. Nonsequential
development or “developmental deviancy”
is commonly observed in children with
developmental disabilities. Children with
more severe forms of CP often have exces-
sive extensor arching of trunk and extremi-
ties during the first few months of life.

This extensor arching frequently leads to
recurrent “flipping episodes” or early roll-
ing before adequate head control in sup-
portive sitting is achieved. True rolling in
both directions usually follows the devel-
opment of head control in normal or typi-
cally developing infants. Similarly, promi-
nent echolalia or the repetition of phrases
or sentences by children and inaccurate
accounts of language milestones can result
in deviant or nonsequential language histo-
ries. For example, a 36-month-old boy was
reported by his parents to have a vocabulary
of 20 words and the use of short sentences.
Normal development of language is charac-
terized by the expression of short three- to
four-word sentences when a vocabulary of
at least 50 to 100 words is achieved. This
particular child was not speaking in original
short three- to four-word sentences but was
engaging in echolalia or “parroting speech.”
Further questioning revealed that the child
was speaking in short phrases, which is
consistent with a vocabulary of 20 words
and a language age of approximately 16 to
18 months.
Neurodevelopmental
Examination
The performance of an accurate and com-
plete physical examination of children at
various developmental ages requires keen
observational skills, good judgment, a
sense of timing, flexibility, and patience.
These skills are not easily mastered and
should continue to be developed and
refined throughout the careers of physi-
cians, nurses, physical and occupational
therapists, speech-language pathologists,
psychologists, educators, and other devel-
opmental professionals. It is important that
all professionals who assess the swallowing
and feeding of young children can perform
a basic neurodevelopmental examination
Table 3–3. Dissociation of Streams of Development Observed in Various
Developmental Disabilities
Streams of
Development
Intellectual
Disabilities
Communication
Disorders
Cerebral
Palsy
Motor
Gross V N D
Fine V N D
Problem-solving (visual) D N V
Language
Expressive D D V
Receptive D V V
Social-adaptive D N D
Note. N = normal; D = delayed; V = variable.
Source: Adapted from Capute, A. J., Accardo, P. J. (1996). A neurodevelopmental perspec-
tive on the continuum of developmental disabilities. In A. J. Capute P. J. Accardo (Eds.),
Developmental disabilities in infancy and childhood (2nd ed., p. 4). Baltimore, MD: Paul H.
Brookes Publishing.

competently that includes key components
of a neurological examination.
The completion of a health and develop-
mental history is often an excellent oppor-
tunity for the child and examiner to get to
know each other before any direct examina-
tion or contact takes place. It is often helpful
to begin the physical examination with the
neurodevelopmental assessment. Initially,
parent–child interactions and reported
developmental milestones can be observed
directly. These observations will help guide
more structured or standardized assess-
ments of cognition and communication
including the Capute Scales (Capute et al.,
1986) and the Bayley Scales of Infant Devel-
opment III (Bayley, 2006). Valid and reliable
measures of general motor development of
infants and toddlers include the Alberta
Infant Motor Scale (AIMS) (Piper, Pinnell,
Darrah, Mahapatra, 1992) and the Pea-
body Developmental Motor Scales (PDMS)
(Hinderer, Richardson, Atwater, 1989).
The Task Force on Infant Position-
ing and SIDS of the American Academy
of Pediatrics recommended in 1992 that
“healthy infants, when being put down for
sleep, be positioned on their side or back.”
Subsequent investigations have demon-
strated that sleep and positioning of awake
infants influence early motor milestones in
the first 15 months. Utilizing the AIMS and
PDMS, investigators have demonstrated
that supine sleep position is associated with
motor delays in the first 15 months and that
increased prone positioning or “tummy
time” improves early motor performance
(Majnemer Barr, 2005, 2006).
Standardized measures of functional or
daily living skills (eating, grooming, dress-
ing, mobility, toileting, and communication
skills) include the Functional Independence
Measure for Children (WeeFIM) (Msall,
Rogers, Ripstein, Lyon, Wilczenski, 1997)
and the Pediatric Evaluation of Disability
Inventory (PEDI) (Haley, Coster, Ludlow,
Haltiwanger, Andrellos, 1992).
Observation of the quality or the way
in which a child performs various motor
skills is an important part of a neurodevel-
opmental examination. Figure 3–1 depicts a
10-month-old boy with delays in both gross
and fine motor skills. In a supported sitting
position, significantly increased hip and
knee flexion can be observed (Figure 3–1A).
When he is pulled to a standing position,
a moderate degree of extensor posturing
and adduction of his legs can be seen (Fig-
ure 3–1B). Excessive finger “splaying” and
adduction of his left thumb can be appre-
ciated during attempts to pick up a small
pellet (Figure 3–1C). Neurologic examina-
tion revealed mild spasticity of the arms and
moderate spasticity of the legs. A diagnosis
of mild CP with spastic diplegia was made.
Examination of Muscle Tone
Examination of muscle tone and resting
posture is an essential part of a neuro-
logic examination. Passive muscle tone is
the amount of resistance that the exam-
iner feels while moving a relatively relaxed
extremity and cannot be determined during
active or voluntary movement of the trunk
or extremities. Depending on the age and
size of the child, passive muscle tone can
be evaluated centrally (in trunk) as well as
peripherally (in extremities). Truncal tone
can be appreciated by suspending the infant
in a prone position. The truncal resistance
and posture during this maneuver can be
observed. Axillary suspension is another
measure of truncal tone. This assessment
consists of holding the infant under the
arms in upright vertical suspension and
documenting whether the infant can sup-
port weight under the arms or whether the
infant “slips through” the examiner’s hands.

Passive flexion and extension of shoulders,
elbows, hips, knees, and ankles can be per-
formed. Range of motion of various joints
can be measured including the anterior
scarf and popliteal angles (Amiel-Tison
Gremier, 1986). The anterior scarf is the
position of the arm at the point of maxi-
mal resistance when it is pulled medially
across the chest with the infant in supine
position. The popliteal angle is formed
Figure 3–1. A. A 10-month-old infant with
cerebral palsy (spastic diplegia). In supported
sitting, excessive hip and knee flexion can be
seen. B. In supported standing, an obligatory
positive support reflex is present. Extensor
posturing and adduction of the legs can be
seen. continues
B
A

when the lower leg is extended to the point
of maximal resistance with the infant in
supine position and the thigh fully flexed
onto the abdomen. The popliteal angle is 0°
when the thigh is fully flexed on the abdo-
menandthelowerlegisfullyextended.A90°
popliteal angle represents a right angle be-
tween the lower leg and thigh.
Muscle tone undergoes developmental
maturation. Passive flexor tone, or resis-
tance to passive extension of an extremity,
develops in a caudal to cephalic fashion in
preterm infants. It first appears in the legs
of preterm infants at about 31 to 32 weeks’
postmenstrual age (PMA) (Allen Capute,
1990). Flexor tone becomes equal in arms
and legs by 35 to 37 weeks’ PMA, and this
persists through 42 weeks PMA (Amiel-
Tison Gremier, 1986). A similar trend
is seen in the popliteal angles. At 31 to 32
weeks’ PMA, the popliteal angles are usually
40° to 60°. By 35 to 42 weeks, the popliteal
angles increase to 90°.
Once established, passive flexor tone
gradually decreases during the 1st year of life
in a cephalic to caudal pattern. By 4 months
of age, full-term infants will have much less
flexor tone in their arms compared with
their legs. Eventually, by 9 months of age,
passive flexor tone should be minimal in
the arms and legs (Amiel-Tison Gremier,
1986). This reduction of passive tone in the
legs is manifested by the typical “feet in the
mouth” posture of normally developing
infants after 7 months of age. By 9 months
of age, popliteal angles have decreased to 30°
to 40°. The resting posture of an infant or
child, in general, reflects passive muscle tone.
A typical posture of a premature infant at 30
to 32 weeks’ PMA is that of arms extended
and legs semiflexed. By 35 to 37 weeks’ PMA,
both arms and legs are equally semiflexed.
Arms tend to be more extended than legs
by 4 months of age; arms and legs tend to be
more extended than flexed by 9 to 12 months
of age (Amiel-Tison Gremier, 1986).
C
Figure 3–1. continued
C. The infant is attempting to pick up a small sugar
pellet with his left hand. Excessive adduction of his left thumb and finger
splaying can be appreciated.

The development of truncal and axillary
muscle tone follows a cranial to caudal pro-
gression in the immediate postnatal years.
By 35 weeks’ PMA, there is an equal dis-
tribution of flexor and extensor tone of the
trunk. This pattern continues throughout
infancy and childhood. This pattern can be
most readily appreciated by observing rest-
ing posture and by the pull-to-sit maneu-
ver. The pull-to-sit maneuver consists of
pulling the infant from supine to a sitting
position. Typically, when pulled to a sit-
ting position, there is an equal tendency
for an infant’s head to either drop forward
or backward. Another useful maneuver in
assessing truncal tone involves the prone
suspension of an infant over the examiner’s
hand. By 35 weeks’ PMA and through-
out the first few years of life, infants will
momentarily align their trunk in a parallel
fashion with the examiner’s hand while held
in a prone position.
Muscle strength, or active muscle tone,
is the amount of resistance the examiner
“feels” when the infant or child actively
resists or moves against gravity. Active or
spontaneous extremity movements against
gravity are often diminished in infants with
significant muscle weakness. Weakness
of facial muscles can result in a “masked
facies.” Traction responses are also quite
helpful in detecting significant weakness.
A traction response represents the amount
of active resistance an infant will use when
a hand or foot is extended manually.
Observation of resting posture is ex-
tremely helpful in assessing muscle tone.
The resting posture of equal extremity flex-
ion in an infant at 36 weeks’ PMA demon-
strates normal flexor extremity tone (Fig-
ure 3–2A). Arm and leg traction responses
indicate normal muscle strength (Figure
3–2B and C). Prone suspension over the
examiner’s hand reveals momentary paral-
lel alignment of the infant’s trunk with the
examiner’s hand, indicating normal truncal
tone (Figure 3–2D). Figures 3–2E–I demon-
strate the degree of head control that can be
expected at this age, as well as the balance
of flexor and extensor tone of the trunk.
In contrast, another preterm infant at 35
weeks’ PMA shows minimal flexion of
extremities and excessive supination of arms
(Figure 3–3A). This infant also had a weak
suck and swallowing problems. A computed
tomography (CT) scan revealed possible
ischemic brain injury. Neurodevelopmental
examination revealed central and peripheral
hypotonia without significant weakness. In
prone suspension, truncal “draping” over
the examiner’s hand and noticeable arm
and leg extension are seen (Figure 3–3B).
Figure 3–3C illustrates the marked increase
in head lag of the infant when pulled to a
sitting position.
Figure 3–2. A. A 36-week postmenstrual age
infant in supine position demonstrating normal
flexor posture of arms and legs. Arms and legs
are about equally flexed. continues
A

93
B–C. The examiner
is demonstrating the
elicitation of the traction
response. Gentle pulling or
extension of the arm (B)
or leg (C) results in active
resistance or flexion of that
extremity. D. The infant is
suspended over the exam-
iner’s hand. Infants after
34 weeks’ postmenstrual
age have sufficient truncal
muscle tone (active and
passive) to briefly (2–3 s)
maintain their trunk and
occiput in line or parallel
with the examiner’s hand
when placed in prone
suspension. continues
C
B
D

94
E–I.This sequence demonstrates the pull-to-sit maneuver.The degree
of head lag is within normal limits for infants from 35 to 42 weeks’ postmenstrual age. Once
the infant is brought to a sitting position, the head drops forward easily, demonstrating a lack of
excessive extensor tone of the trunk.
I
G H
E F

The initial reduction of passive flexor
tone of the upper extremities during the
first few postnatal months is demonstrated
in Figure 3–4. This infant is a 26-week-
gestation preterm infant at a corrected age
of 3 months. Reassuring findings include
midline position of the head, with legs semi-
flexed and arms extended. Examination was
normal, revealing diminished passive flexor
tone of the arms compared to the legs.
This normal pattern of passive muscle
tone and posture is in contrast to observa-
tions of a 3-month-old infant with known
brain injury (Figure 3–5). Figure 3–5A
demonstrates excessive lateral turning of
the head and equal flexor posture of the
arms and legs. Excessive extensor arching of
the trunk is demonstrated in Figure 3–5B.
Examination was abnormal and confirmed
equal amounts of passive flexor tone of
the arms and legs and extensor tone of the
trunk and extremities. Increased truncal
extensor tone can also be appreciated by the
pull-to-sit maneuver. As early as 3 months,
abnormal asymmetries of muscle tone and
strength can be appreciated. Figure 3–6A
reveals a 3-month-old term infant with a
history of dysphagia and gastroesophageal
reflux in a supported sitting position on his
father’s lap. This infant holds his left arm in
an overly flexed posture when compared to
his right arm. His left hand is held in a fisted
posture when compared to his right hand.
This same pattern of upper extremity rest-
ing posture can be seen in prone position
(Figure 3–6B). Neurological examination
revealed increased passive muscle tone and
reduced spontaneous movements of his left
arm and leg, and popliteal angles that were
60° on the right, and 90° on the left.
Figure 3–3. A. The supine, resting posture of a 35-week
postmenstrual age infant with diffuse hypotonia (reduced pas-
sive muscle tone). Minimal flexion of the extremities is evident
compared with posture of the infant in Figure 3–2A. continues
A

96
B. In prone suspension, the infant’s trunk is draped
excessively over the examiner’s hand. C. Infant being pulled from supine to
a sitting position, demonstrating excessive head lag.
C
B

97
Figure 3–4. This infant is a 26-week preterm at 3 months corrected age. In
supine position, this infant demonstrates a normal supine resting posture for
a 3-month-old infant. Head is midline, arms are held in an extended position,
and legs are still semiflexed.
Figure 3–5. A. A 3-month-old infant with known brain injury is viewed in supine position. Arms
and legs are held in a flexed position, similar to a normal newborn posture. Excessive lateral
head turning and truncal extensor arching can be appreciated. B. Excessive truncal extensor
arching can be seen while the infant is lying on his side.
A B

Figure 3–7A shows a former 30-weeks’
gestation preterm infant at 6 months cor-
rected age in supine position. The normal
resting posture reveals arms relaxed in
extension and hips and knees held in semi-
flexion. Additionally, this infant demon-
strates the normal degree of hip adduction
and mild degree of foot dorsiflexion. Figure
3–7B is a lateral view that demonstrates a
normal popliteal angle of approximately 60°.
Typically, infants at this age can successfully
be vertically suspended under their axillae
as shown in Figure 3–7C.
The following figures provide further
examples of abnormalities of passive muscle
tone that are frequently observed in infants
with neurogenic dysphagia. Figure 3–8A
reveals a 4-month-old infant who had a
history of choanal stenosis, laryngoma-
lacia, and neonatal dysphagia eventually
requiring a gastrostomy tube. On exami-
nation, this infant had diffuse hypotonia
in the presence of normal range muscle
strength. In the supine view, the infant’s
arms are semiflexed, and hips are exces-
sively abducted and semiextended, which
is a posture commonly seen in infants with
hypotonia. On vertical axillary suspension,
the infant is seen “slipping through” in Fig-
ure 3–8B. These findings are consistent with
CNS-based hypotonia.
CNS Evaluation
A common finding in infants with central
nervous disorders affecting motor devel-
opment is the simultaneous presence of
Figure 3–6. A. A 3-month-old term infant holds his left arm in an overly flexed posture when
compared to his right arm. His left hand is held in a fisted posture when compared to his right
hand. B. In prone position, this infant holds his left arm in more flexion and against his chest
when compared to his right arm.
A B

99
Figure 3–7. A. A 6-month corrected age former 30-week gestation preterm infant in supine
position. The normal resting posture reveals arms relaxed in extension, and hips and knees
held in semiflexion. Noteworthy is the infant’s normal degree of hip adduction and mild degree
of foot dorsiflexion at rest. B. In this lateral view the examiner demonstrates a normal popliteal
angle of approximately 60° at 6 months corrected age. C. The examiner suspends the infant
under the arms and demonstrates the normal absence of axillary “slip through.”
C
A B

truncal hypotonia, and extensor tone of
the trunk and extremities. Figure 3–9A
reveals the resting posture of a 32-weeks’
gestation preterm infant at 6 months cor-
rected age. This infant’s hips are overly
adducted. Legs and feet are held in exten-
sion. On examination, this infant’s heel
cords were held in tight extension and there
was increased resistance to hip abduction.
Figure 3–9B reveals the examiner meeting
significant resistance to dorsiflexion of the
foot. The popliteal angle in Figure 3–9C
was approximately 90° indicating increased
hamstring muscle tightness. Generally by
this age, popliteal angles should be 60° or
less as previously demonstrated in Figure
3–7B. Figure 3–9D reveals this infant in
a supported standing position with knees
overly extended, and “tip toe” or equinus
posture of feet. Figure 3–10 illustrates the
presence of excessive trunk extensor tone
in a 6-month-old infant who had just been
brought to a sitting position. Typically at
this age, infants should have no head lag
when pulled to sit, and balance between
truncal flexor and extensor tone when in a
supported or independent sitting position.
This figure highlights the presence of trun-
cal extensor tone that is common in infants
with abnormalities of early brain develop-
ment affecting motor development.
CNS disorders are usually character-
ized by abnormalities of muscle tone in
the absence of significant muscle weakness
(decreased active tone). Common abnor-
malities of passive muscle tone include early
hypotonia, persistence of significant flexor
tone of the extremities after 9 months of
age, and the presence of excessive extensor
tone of the trunk and extremities. Children
Figure 3–8. A. A 4-month-old infant with hypotonia can be observed in supine with arms semi-
flexed, and hips are excessively abducted and semiextended. B. Vertical axillary suspension of
this infant demonstrates “axillary slip through.”
A B

101
Figure 3–9. A. A hypertonic 32-weeks’gesta-
tion preterm infant, at 6 months corrected age.
Hips are overly adducted, and legs and feet
are held in held in extension. B. The examiner
attempting to dorsiflex the foot to 90° but meet-
ing significant resistance. C. Increased ham-
string muscle tone is demonstrated by a 90°
popliteal angle. D. Same infant in a supported
standing position with knees overly extended,
and “tip toe” or equinus posture of feet.
D
A
B
C

with neuromuscular disorders including
anterior horn cell diseases, myopathies, and
muscular dystrophies generally have rather
significant reductions in active muscle tone
(muscle strength) and less striking reduc-
tion in passive muscle tone. Table 3–4 con-
tains key physical examination findings that
are useful in differentiating disorders of the
CNS from neuromuscular conditions that
result in swallowing and feeding problems.
CP is a condition that results from a
static nonprogressive cerebral lesion that
occurs during the developmental period of
early childhood and is manifested by motor
delays, abnormal neuromotor findings, and
a high prevalence of other CNS deficits in
areas of cognition and neurobehavior. CP
is one of the most common causes of neu-
rogenic dysphagia. The resting posture of a
27-month-old girl with CP reveals excessive
flexion of her arms and predominant exten-
sor posturing of her legs (Figure 3–11A).
These findings reflect increased passive
flexor tone of the arms and passive exten-
sor tone of the legs. Figure 3–11B illustrates
the normal resting posture of children after
the first 9 to 12 months of life. Typically,
passive flexor tone of the arms and legs has
significantly decreased, and the resulting
posture consists of only minimal flexion of
the extremities. Passive extensor tone and
posture of the extremities should not be
observed at any age. These abnormalities
can also be appreciated by the pull-to-sit
maneuver seen in Figure 3–11C–E. This
type of extensor posturing can result in
the child coming to a stand rather than to
a sit. Abnormally increased passive muscle
tone in the lower extremities usually results
in popliteal angles of 90° or more (Figure
3–11F). A common finding in children with
cerebral injuries including CP is the “cor-
tical thumb” (Figure 3–11G). This find-
ing consists of the adduction of the thumb
across the palm.
An 8-month-old girl with myotonic dys-
trophy provides further illustrations of find-
ings important in a physical examination.
Figure 3–10. A 6-month-old infant with excessive neck and trunk extensor
tone has been brought up to a sitting position. Extensor posturing of the neck
and trunk can be appreciated.

103
Figure 3–11. A. This 27-month-old girl with cerebral palsy has a resting posture that reflects
her abnormal passive muscle tone. Her arms are excessively flexed, her hands are fisted, and
her legs are extended. She had persistent moderate passive flexor tone of her arms and exten-
sor tone of her legs. B. The normal reduction of passive flexor tone in the first 9–12 months
can be appreciated by the posture of this 1-year-old child. Arms and legs are positioned with
minimal flexion. continues
A B
Table 3–4. Differentiation of Central Nervous System and Neuromuscular Causes of
Dysphagia in Childhood
Neurologic Examination
Central Nervous
System Disorders Neuromuscular Disorders
Passive tone Variable, hypertonia is
common
Hypotonia
Active tone (strength) Normal or mildly decreased Significantly decreased
Deep tendon reflexes Normal or increased Decreased or absent
Primitive reflexes Usually strong and
persistent
May be absent or normal in
degree and duration
Plantar response Commonly up-going Plantarflexion (+ Babinski)
Cognition Cognitive deficits Usually normal

104
Figure 3–11. continued C–E. Extensor tone of the trunk and legs can be appreciated by the
pull-to-sit maneuver. Pulling this 27-month-old girl from supine to sitting results in extensor
posturing of the trunk and legs and coming to stand rather than to sit. F. This popliteal angle of
approximately 90° indicates increased hamstring muscle tone. G. The persistent adduction of
the thumb across the palm (cortical thumb) is abnormal at any age.
G
E F
C D

She has a typical myopathic facies (inverted
V posture of the mouth) and extension of
the extremities (Figure 3–12A). In sup-
ported sitting, a collapsing kyphosis can be
seen (Figure 3–12B). Physical examination
revealed minimal spontaneous extremity
movements against gravity. Active muscle
tone was severely diminished compared
with the reduction of passive muscle tone.
Figure 3–12C is a CT scan of her brain
Figure 3–12. A. An 8-month-old girl with myotonic dystrophy. In supine posi-
tion, notable features include extension of arms and legs, and myopathic
facies (inverted “V” shape of her mouth). B. In supported sitting, a collapsing
kyphosis can be seen. continues
B
A

revealing ventricular and extra-axial promi-
nence, suggestive of cerebral atrophy. These
brain anomalies are common in patients
with myotonic dystrophy.
Spontaneous movements of infants refer
to endogenously generated motor activi-
ties. These generalized movements (GMs)
are sensitive indicators of brain function
(Einspieler, Prechtl, Ferrari, Cioni, Bos,
1997). GMs emerge during early fetal life
(deVries, Visser, Prechtl, 1982) and dis-
appear around 3 to 4 months postterm
when goal-directed motor behavior begins
(Hadders-Algra Prechtl, 1993; Hopkins
Prechtl, 1984). Normal GMs show age-
dependent characteristics. Before 36 to 38
weeks’ PMA, GMs are characterized by an
enormous variation in movement trajec-
tory, speed, and amplitude (Hadders-Algra
Prechtl, 1993). Generalized movements
develop a writhing character that is slower
and more powerful than preterm GMs.
At the end of the 2nd postterm month,
writhing movements are replaced by GMs
that have a fidgety character. Fidgety GMs
consist of a continuous stream of tiny, ele-
gant movements occurring irregularly over
the entire body. Normal GMs at any age
are characterized by their complexity, vari-
ability, and fluency. In contrast, abnormal
GMs show a reduced complexity, variabil-
ity, and fluency (Prechtl, 1990). GMs have
been shown to be one of the best predic-
tors of neurologic outcomes in high-risk
infants (Cioni et al., 1997). A recent system-
atic review of tests to predict CP in young
children revealed that general movement
assessment (absence of fidgety movements
or presence of cramped synchronized gen-
eral movements) is the most sensitive and
specific test currently available that pre-
dicts which infants develop CP (Bosanquet,
Copeland,Ware,Boyd,2013;Morgan et al.,
2016).
C. A computed tomography scan of her brain
revealed ventricular and extra-axial prominence, suggestive of cerebral
atrophy.
C

Evaluation of deep tendon reflexes is
helpful in children with dysphagia and
abnormalities of motor development. Most
children with CNS disorders associated
with abnormalities of motor development
have increased or normal deep tendon re-
flexes. Neuromuscular disorders involving
the anterior horn cells of the spinal cord,
peripheral nerves, or muscles usually result
in decreased or absent deep tendon reflexes.
Characteristic of CNS disorders, particu-
larly CP, is the persistence of strong primitive
reflexes. A useful manual for the assessment
of primitive reflexes has been compiled by
Capute (1979). These are brain-stem-medi-
ated reflexes that are most prominent in the
first 3 months of life and generally disappear
by 6 to 9 months of age. The most clinically
useful reflexes include the Moro, tonic laby-
rinthine, asymmetric tonic neck, and posi-
tive support reflexes. These reflexes can be
elicited by various maneuvers of the head
and neck, and persistence of these reflexes
generally precludes the development of
higher voluntary motor skills including sit-
ting, crawling, and walking (Figure 3–13).
Primitive reflexes are important determi-
nants of proper positioning during feeding
in children with CNS disorder. Figure 3–14A
and B demonstrate elicitation of the tonic
labyrinthine reflex in a 27-month-old girl.
Extension of her neck results in shoulder
retraction and trunk and leg extension;
when her neck is flexed, protraction of the
shoulders and hip flexion results. Figure
3–14C illustrates the difficulties this child’s
mother had trying to position her for feed-
ing in the presence of a strong tonic labyrin-
thine reflex. Proper positioning techniques,
including supportive neck and hip flexion,
dramatically improved her positioning dur-
ing feeding (Figure 3–14D). The asymmet-
ric tonic neck reflex or the “fencer posture”
can also have a deleterious effect on posi-
tioning during feeding. A strong or obliga-
tory asymmetric tonic neck reflex often will
result in asymmetric sitting posture during
feeding (Figure 3–15).
Cranial Nerve (CN) Examination
A detailed examination of the cranial nerves
involved with swallowing can be completed
in most children. The trigeminal (CN V),
facial (CN VII), glossopharyngeal (CN IX),
vagus (CN X), and hypoglossal (CN XII)
cranial nerves control the sensory and
motor components of swallowing. Their
nuclei are located in the pontomedullary
area of the brain stem. A detailed discus-
sion of the neural control of deglutition is
presented in Chapter 2.
A summary of physical examina-
tion findings useful in localizing the site
of cranial nerve deficits that may be seen
in patients with dysphagia is presented in
Table 3–5. Supranuclear lesions are located
in cerebral or descending efferent path-
ways above or proximal to the cranial
nerve nucleus in the brain stem. Nuclear or
peripheral lesions are located in the cranial
nerve nucleus in the brain stem or periph-
eral pathways from the brain stem to the
target organ. Generally, supranuclear lesions
can result in disordered movement without
paralysis, atrophy, or signs of denervation
including fasciculations. Nuclear or periph-
eral lesions, in contrast, result in significant
paresis or paralysis, muscle atrophy, and
fasciculations. Nuclear or peripheral cranial
nerve deficits in patients with dysphagia
usually result from acute traumatic or isch-
emic injuries, destructive lesions (tumors,
syringobulbia), or infections (polio, acute
inflammatory polyradiculoneuropathy)
that in turn are of acute diagnostic signifi-
cance to the clinician.

108
Figure 3–13. A. The Moro reflex can be elicited with the infant in supine position. The infant’s
head is allowed to drop back suddenly from at least 3 cm off a padded surface (1). On extension
of the neck, there is a quick symmetrical abduction and upward movement of the arms followed
by opening of the hands (2). Adduction and flexion of the arms can then be noted. B. The tonic
labyrinthine reflex can be evaluated in supine or prone position. In supine, the infant’s head is
extended 45° below the horizontal and then flexed 45° above the horizontal. While the neck
is extended 45°, the limbs are extended (1). With the neck flexed 45°, the limbs are flexed (2).
C. The asymmetric tonic neck reflex can be elicited by turning an infant’s head laterally when
in a supine position. Visible evidence includes extension of the extremities on the chin side or
flexion on the occiput side. D. A positive support reflex can be elicited by suspending the infant
around the chest. The infant is bounced five times on the balls of his feet. The balls of the feet
are then brought in contact with the table surface. Co-contraction of opposing muscle groups of
the legs occurs, resulting in a position capable of supporting weight.

109
Figure 3–14. A. This 27-month-old girl demonstrates the presence of a strong tonic labyrin-
thine reflex. Mild extension of her neck results in shoulder retraction and leg extension. B. Neck
flexion results in protraction (forward placement) of her shoulders and arms and leg flexion.
This posture is generally more conducive for oral feeding. C. This 27-month-old girl with cere-
bral palsy is being held on her mother’s lap in preparation for an oral feeding. The strong tonic
labyrinthine reflex results in excessive trunk and neck extension which may increase the risk for
aspiration. D. Proper positioning including hip and knee flexion and flexion of the neck results
in a more favorable position for feeding.
C
A B
D

110
Figure 3–15. This 27-month-old girl demon-
strates the presence of a strong asymmet-
ric tonic neck reflex. Her arms and legs are
extended in the direction in which her head is
turned and flexed on the occiput side.
Table 3–5. Clinical Localization of Cranial Nerve Deficits Associated With Dysphagia
Cranial Nerve Supranuclear Lesions Nuclear or Peripheral Lesions
Trigeminal (CN V) • Mandible movements are well
preserved but often immature
or poorly coordinated
• Mandible movements are
usually minimal or absent
• Jawreflexpresentorexaggerated • Jaw reflex is usually absent
• Tonic bite reflex may be present • Tonic bite reflex is absent
Facial Nerve
(CN VII)
• Paralysis of lower half of face.
Paralysis is almost always
unilateral
• Paralysis of upper (forehead)
and lower half of face;
paralysis can be unilateral or
bilateral
Glossopharyngeal
(CN IX) and
vagus nerves
(CN X)
• Muscular palate has normal
strength
• Weakness of muscular palate;
asymmetry of muscular palate
movement is common
• Normal appearance of
palatopharyngeal folds
• Flattening or asymmetry of
palatopharyngeal folds
• Vocal fold movement preserved • Vocal fold paralysis
Hypoglossal
nerve (CN XII)
• Tongue movements are
dysfunctional but present
• Unilateral or bilateral absence
of tongue movements
• Tongue protrusion reflex can be
exaggerated and prolonged in
duration
• Tongue fasciculations
• Tongue atrophy

The trigeminal nerve (CN V) contains
motor and sensory fibers important in bolus
formation and oral transit phase of swal-
lowing. The third (mandibular) division of
CN V innervates the muscles of mastication
(temporalis, masseters, and pterygoids).
Nuclear lesions of the trigeminal nerve will
produce drooping or opening of the jaw and
absence of the jaw reflex (jaw jerk). Atro-
phy of the temporalis and masseter muscles
may be seen. Supranuclear lesions will result
in poorly coordinated jaw movements and
exaggerated jaw reflex. Sensory fibers for
pinprick and light touch are provided to the
mucous membranes of the nose and mouth;
sensation is also provided to the face in a
similarly complex manner.
The facial nerve (CN VII) innervates
muscles of facial expression. The seventh
nerve nuclei are located in the pons. The
rostral part of the nucleus controls the ipsi-
lateral forehead and the caudal part controls
the ipsilateral cheek. The rostral part of the
facial nucleus is controlled by pyramidal
tracts from both cerebral hemispheres.
Bilateral supranuclear damage is required
to produce paralysis of the forehead mus-
culature.When paralysis is of central origin,
however, weakness of the lower facial mus-
culature is commonly found with contralat-
eral pyramidal tract lesions superior to the
facial nucleus. These types of lesions will
be manifested by contralateral widening of
the palpebral fissure and flattening of the
nasolabial fold. The facial nerve also pro-
vides motor fibers to the stylohyoid muscle
and posterior belly of the digastric muscle.
Paralysis of the facial nerve may lead to
abnormalities of the pharyngeal swallow
resulting in delayed passage of food (Bass,
1988). Parasympathetic innervation to the
salivary glands and mucous membranes
travels with CN VII and controls produc-
tion of saliva (Chapter 11). Sensory fibers
provide taste sensation for the anterior two
thirds of the tongue.
The glossopharyngeal nerve (CN IX)
provides motor innervation to the sty-
lopharyngeus muscle and sensory fibers
to the mucous membranes of the inferior
aspect of the muscular palate, mucosa of the
tongue, and the posterior pharyngeal wall.
The nuclei of the glossopharyngeal and
vagus nerves are represented by the saliva-
tory nuclei, the nucleus tractus solitarius,
and the nucleus ambiguus of the medulla.
The sensory component of the gag reflex is
carried by CN IX, and the motor output for
the gag reflex includes the vagus, hypoglos-
sal, and trigeminal nerves. The most effec-
tive receptor regions for the elicitation of the
pharyngeal phase of swallowing are inner-
vated by fibers of CN IX carried through
the pharyngeal plexus and by the superior
laryngeal branch (SLN) of CN X. A delay in
swallow initiation is one of the more com-
mon abnormalities in children with dyspha-
gia and CP (Rogers, Arvedson, Buck, Smart,
Msall, 1994).
Motor nerve fibers are provided to the
soft palate, pharynx, and larynx by CN X.
The dorsal motor nucleus of the vagus
nerve, located in the medulla, contributes
to the sensorimotor integration of swallow-
ing, respiration, phonation, cardiovascular
responses, and emesis. An acute unilateral
lesion of the vagus nerve or its nucleus in
the dorsolateral part of the medulla may
result in neurogenic dysphagia, with ipsi-
lateral weakness of the muscular palate,
asymmetry of the palatopharyngeal folds,
and ipsilateral vocal fold paralysis.
The hypoglossal nerve (CN XII) is
involved with complex movements of the
tongue that have impact on hyoid and lar-
ynx function, all of which are important for
normal swallowing. Injury to the hypoglos-
sal nerve or to its nucleus in the medulla

leads to loss of muscle mass on the ipsilat-
eral side of the tongue. Unilateral weakness
of the extrinsic muscles of the tongue results
in protrusion to the weak side. Supranuclear
lesions characteristic of CP result in weak-
ness, without muscle atrophy or fascicula-
tions, as well as poorly coordinated tongue
movements.
A 26-week gestation, preterm male infant
at 41 weeks’ PMA is seen in Figure 3–16.
Perinatal history was significant for severe
asphyxia. Prolonged mechanical ventilation
and nasogastric feedings were required.
A feeding gastrostomy tube was subse-
quently placed. In supine position while
crying vigorously, a “mask-like” facies is
seen, consistent with bilateral nuclear facial
(CN VII) paralysis (Figure 3–16A). The
jaw is wide open with no active movement,
consistent with bilateral trigeminal nerve
(CN V) paralysis. He could not suckle
and had a very weak cry. A close-up facial
view reveals no spontaneous movements
of his tongue (Figure 3–16B). His tongue
appeared atrophied and somewhat asym-
metric. A subsequent laryngoscopy revealed
bilateral vocal fold paralysis. Bilateral exten-
sor tone was observed in his legs. Audio-
logic evaluation revealed bilateral senso-
rineural hearing loss. The etiology for the
wide range of deficits was severe perinatal
asphyxia with patchy ischemic necrosis of
his brain stem involving cranial nerves V,
VII, VIII, IX, X, and XII, as well as cortico-
spinal tract dysfunction.
Further examples of the physical find-
ings associated with supranuclear and
nuclear lesions of the hypoglossal (CN XII)
nerve can be found in Figure 3–17. Fig-
ure 3–17A shows the prominent tongue
thrust reflex in a 27-month-old girl with CP.
Reflexive protrusion of the tongue is spon-
taneous and on examination poorly con-
trolled tongue movements are associated
with normal tongue mass. A young boy with
spina bifida is pictured in Figure 3–17B.
He was diagnosed with an Arnold-Chiari
malformation and received a ventricular
peritoneal shunt in the newborn period.
The type II Arnold-Chiari malformation
consists of downward displacement or her-
niation of the brain stem (medulla) and
cerebellar tonsils through the foramen mag-
num. This type of malformation is common
in patients with spina bifida and can result
in brain-stem dysfunction. Figure 3–17C
demonstrates the patient’s asymmetric
tongue atrophy. Tongue fasciculations were
also evident.
Dysmorphology Evaluation
Recognized genetic syndromes, particu-
larly those associated with developmental
disabilities, are important causes of neu-
rogenic dysphagia. These conditions are
generally recognized by a careful search for
major or minor malformations. The more
common genetic syndromes associated with
dysphagia are listed in Table 3–6. References
that were used heavily included the web-
sites Online Mendelian Inheritance in Man
(OMIM) and GeneReviews. See Chapter 12.

113
Figure 3–16. A. A 26-week gestation preterm
infant at 41 weeks’ PMA in supine position
during a crying episode. “Mask-like” facies is
evident. B. A closer view of the face reveals
asymmetric tongue atrophy.
B
A

114
Figure 3–17. A. Prominent tongue thrusting
or protrusion is evident in this 27-month-old
girl with cerebral palsy. Tongue mass is pre-
served. These findings are commonly found
in suprabulbar or supranuclear palsy of the
hypoglossal cranial nerve. B. This 6-year-old
boy has asymmetric tongue atrophy resulting
from an Arnold-Chiari malformation with spina
bifida. Tongue atrophy is most consistent with
a bulbar nuclear lesion of the hypoglossal cra-
nial nerve.C. Close-up of child in Figure 3–17B
showing asymmetric tongue atrophy.
B
A
C

115
Table
3–6. Common
Genetic
Conditions
With
Prominent
Dysphagia
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Noonan
syndrome
12q24.13
•
Short
stature
•
Congenital
heart
defects
•
Broad
forehead
•
Hypertelorism
•
Downward
palpebral
fissures
•
A
high-arched
palate
•
Low-set,
posteriorly
rotated
ears
•
Webbed
neck
•
Language
delays
•
Articulation
abnormalities
•
Low
average
intelligence,
often
need
special
education
resources
•
Poor
suck
•
Food
refusal
•
Gastroesophageal
reflux
•
Constipation
Costello
syndrome
11p15.5
•
Coarse
facies
•
Postnatal
growth
failure
•
Macrocephaly
•
Curly
or
sparse,
fine
hair
•
Redundant
skin
of
the
neck,
palms,
soles,
and
fingers
•
Congenital
heart
defects
•
Hypotonia
•
Intellectual
disability
•
Severe
feeding
problems
resulting
in
failure
to
thrive
Russell-Silver
syndrome
11p15.5
and
chromosome
7
•
Intrauterine
growth
retardation/
small
for
gestational
age
(10th
percentile)
•
Postnatal
growth
with
height/
length
third
percentile
•
Normal
head
circumference
(3rd–97th
percentile)
•
At
significant
risk
for
developmental
delay
(both
motor
and
cognitive)
and
learning
disabilities
•
Poor
appetite
•
Fussiness
•
Slow
feeding
•
Problems
associated
with
oral-motor
dysfunction
continues

116
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Russell-Silver
syndrome
continued
•
Limb,
body,
and/or
facial
asymmetry
•
Triangular
facies
characterized
by
broad
forehead
and
narrow
chin
•
Fifth-finger
clinodactyly
Kabuki
syndrome
12q13.12,
Xp11.3
•
Failure
to
thrive/postnatal
growth
deficiency
•
Typical
facial
features
(elongated
palpebral
fissures
with
eversion
of
the
lateral
third
of
the
lower
eyelid)
•
Arched
and
broad
eyebrows
•
Short
columella
with
depressed
nasal
tip
•
Large,
prominent,
or
cupped
ears
•
Minor
skeletal
anomalies
•
Persistence
of
fetal
fingertip
pads
•
Hypotonia
•
Majority
have
intellectual
disability
•
Language
delays,
dysarthria,
or
dyspraxia,
seizures
•
Problems
with
suck,
swallow,
gastroesophageal
reflux,
aspiration
pneumonia,
and/or
failure
to
thrive
are
described
in
over
half
of
infants
Table
3–6.
continued

117
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
22q11deletion
syndrome
(also
known
as
Velocardiofacial
syndrome,
DiGeorge
syndrome)
22q11.2
•
Congenital
heart
disease
•
Palatal
anomalies
•
Tubular
nose
•
Narrow
palpebral
fissures
•
Recessed
mandible
•
Small
open
mouth
•
Immune
deficiency
•
Hypocalcemia
•
Developmental
delay,
particularly
language
delay,
intellectual
disability,
and
learning
differences
(nonverbal
learning
disability
where
the
verbal
IQ
is
significantly
greater
than
the
performance
IQ)
are
common
•
Autism
or
autistic
spectrum
disorder
is
found
in
approximately
20%
of
children,
and
psychiatric
illness
(specifically
schizophrenia)
is
present
in
25%
of
adults
•
Trouble
coordinating
the
suck/swallow/breath
pattern,
interrupted
by
gagging
or
regurgitation
•
Recurrent
vomiting
and
constipation
are
common
•
Immature
oral
transport
pattern
•
VFSS
studies
demonstrate
pharyngeal
hypercontractility,
cricopharyngeal
prominence,
and/or
diverticula
X-Linked
Opitz
G/
BBB
syndrome
Xp22.2
•
Ocular
hypertelorism
•
Prominent
forehead,
widow’s
peak,
broad
nasal
bridge,
anteverted
nares
•
Laryngotracheoesophageal
defects
•
Genitourinary
abnormalities
(hypospadias,
cryptorchidism,
and
hypoplastic/bifid
scrotum)
•
Cleft
lip
and/or
palate
are
present
in
approximately
50%
of
affected
individuals
•
Developmental
delay
and
intellectual
disability
are
observed
in
about
50%
of
affected
males
•
Swallowing
problems
with
aspiration
continues

118
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Prader-Willi
syndrome
15q11.2
•
Facial
features
including
narrow
bifrontal
diameter,
almond-
shaped
palpebral
features
•
Small
hands
and
feet
•
Obesity
after
first
year
•
Short
stature
•
Hypogonadotropic
hypogonadism
•
Diminished
fetal
activity
•
Hypotonia
•
Majority
have
mild
intellectual
disability
•
Sucking
and
swallowing
difficulties
most
prominent
in
first
year
but
can
persist
throughout
adulthood
and
associated
with
hyperphagia
and
choking
risk
Coffin-Siris
syndrome
One
of
six
genes
(ARID1A,
ARID1B,
SMARCA4,
SMARCB1,
SMARCE1,
and
SOX11)
•
Aplasia
or
hypoplasia
of
the
distal
phalanx
or
absence
of
the
nail,
typically
involving
the
fifth
finger,
but
other
digits
may
also
be
affected
•
Toes
can
also
be
affected,
where
the
finding
tends
to
involve
multiple
digits
•
Appearance
of
facial
coarseness
including
wide
mouth
with
thick,
everted
upper
and
lower
lips
•
Broad
nasal
bridge
with
broad
nasal
tip,
thick
eyebrows,
and
long
eyelashes
•
Sparse
scallop
hair
particularly
in
infancy,
hirsutism
•
Almost
all
infants
have
developmental
delays
and
hypotonia
•
Oropharyngeal
dysphagia
Table
3–6.
continued

119
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Hallermann-Streiff
syndrome
Sporadic,
none
•
Brachycephaly
with
frontal
bossing
•
Hypotrichosis
•
Microphthalmia
•
Cataracts
•
Beaked
nose
•
Micrognathia
•
Upper
airway
obstruction
•
Skin
atrophy
•
Dental
anomalies
•
Proportionate
short
stature
•
Generally
normal
intelligence
•
Feeding
and
respiratory
problems
common
in
infancy
Smith-Lemli-Opitz
syndrome
11q13.4
(deficiency
of
7-dehdrocholesterol
reductase)
•
Narrow
forehead
•
Epicanthal
folds
•
Ptosis
•
Short
mandible
with
preservation
of
jaw
width
•
Short
nose,
anteverted
nares
•
Low-set
ears
•
2-3
syndactyly
of
the
toes
(minimal
to
Y-shaped)
•
Microcephaly
•
Growth
retardation/short
stature
•
Hypospadias
•
Cleft
palate
•
Postaxial
polydactyly
•
Hypotonia
and
hypertonia
•
Generally
moderate
to
severe
intellectual
disability
•
Oral
hyposensitivity
or
hypersensitivity
and
dysphagia
•
Majority
experience
failure
to
thrive
continues

120
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Cornelia
de
Lange
syndrome
Heterozygous
pathogenic
variant
•
Synophrys
•
Highly
arched
eyebrows,
long
eyelashes
•
Short
nose
with
anteverted
nares
•
Small
widely
spaced
teeth
•
Microcephaly
•
Growth
retardation
(prenatal
onset
5th
centile
throughout
life)
•
Hirsutism
•
Upper
limb
reduction
defects
•
Development
is
typically
delayed,
with
a
range
from
borderline
IQ
with
learning
disabilities
to
profound
mental
retardation,
although
individuals
with
IQ
in
the
normal
range
have
been
seen
•
Many
have
hyperactivity,
short
attention
span,
attention
deficit
disorder
with
or
without
hyperactivity
(ADHD)
•
Aggression,
defiance,
self-
injurious
behavior
•
Extreme
shyness,
perseveration,
obsessive–
compulsive
behaviors,
and
depression
•
Dysphagia
is
common
•
Aspiration
pneumonia
Dubowitz
syndrome
Autosomal
recessive
•
Postnatal
growth
retardation
•
Microcephaly
•
Sloping
forehead
•
Broad
nasal
bridge
•
Small
facies
•
Shallow
supraorbital
ridge
•
Broad
nasal
tip
•
Short
palpebral
fissures
•
Majority
have
intellectual
disability
•
Behavioral
problems
•
Dysphagia
•
Gastroesophageal
reflux
Table
3–6.
continued

121
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Pierre
Robin
(nonsyndromic)
17q24.3-q25.1
•
Mandibular
hypoplasia,
cleft
secondary
palate,
and
glossoptosis
leading
to
life-
threatening
obstructive
apnea
•
Generally
normal
development
•
Respiratory
distress
with
oral
feeding
Hemifacial
microsomia
syndrome
14q32
•
Facial
asymmetry
resulting
from
maxillary
and/or
mandibular
hypoplasia
•
Preauricular
or
facial
tags
•
Ear
malformations
that
can
include
microtia
(hypoplasia
of
the
external
ear)
•
Anotia
(absence
of
the
external
ear)
or
aural
atresia
(absence
of
the
external
ear
canal)
and
hearing
loss
•
Language
delays,
at
risk
for
academic
underachievement
•
Difficulties
with
coordination
of
breathing
and
swallowing
Mobius
sequence
13q12.-q13,
most
are
sporadic
•
Congenital,
nonprogressive
facial
weakness
with
limited
abduction
of
one
or
both
eyes
•
Additional
features
can
include
hearing
loss
and
other
cranial
nerve
dysfunction,
as
well
as
motor,
orofacial,
musculoskeletal,
neurodevelopmental,
and
social
problems
•
Most
have
normal
intelligence,
may
be
at
higher
risk
for
intellectual
disability
•
Speech
problems
are
reported
to
be
common
as
well
as
language
delays
•
Feeding
problems
during
the
first
weeks
or
months
after
birth
are
very
common;
can
be
due
to
insufficient
sucking
or
swallowing,
palatal
weakness,
or
regurgitation
and
can
result
in
poor
growth;
quite
often,
tube
feeding
or,
alternatively,
the
Haberman
Feeder,
may
be
necessary
continues

122
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Rubinstein-Taybi
syndrome
16p13.3
•
Postnatal
growth
deficiency
•
Microcephaly
•
Broad
thumbs
and
halluces
•
Facial
anomalies
including
highly
arched
eyebrows,
long
eyelashes,
downslanting
•
Palpebral
fissures
•
Broad
nasal
bridge,
beaked
nose
with
the
nasal
septum
•
Highly
arched
palate
•
Mild
micrognathia
and
characteristic
grimacing
or
abnormal
smile
•
Affected
individuals
also
have
an
increased
risk
of
tumor
formation
•
Intellectual
disability
is
characteristic
but
there
are
exceptions
•
Behavior
is
otherwise
characterized
by
short
attention
span
and
poor
coordination,
and
in
early
adulthood
sudden
mood
changes
occur
•
Nutritional/feeding
problems
and
gastroesophageal
reflux
associated
respiratory
problems
are
common
•
Some
adolescents
develop
dysphagia,
some
with
esophageal
pathology
(strictures,
post-cricoid
webs,
vascular
rings)
Beckwith-
Wiedemann
syndrome
Imprinted
genes
within
the
chromosome
11p15.5
region
•
Neonatal
macrosomia
•
Postnatal
overgrowth
•
Abdominal
wall
defects
•
Macroglossia
•
Ear
anomalies
•
Nevus
flammeus
•
Hemihyperplasia
•
Neurodevelopment
is
usually
normal
•
Paternally
derived
11p15.5
duplications
are
typically
associated
with
intellectual
disability
•
Macroglossia
can
interfere
with
breathing,
eating,
and
speech
development
needing
surgical
correction
at
times
Table
3–6.
continued

123
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Beckwith-
Wiedemann
syndrome
continued
•
Organomegaly
•
Nephroureteral
malformations
•
Hypoglycemia
•
Predisposition
to
develop
embryonic
tumors
in
infancy
Trisomy
18
•
Prenatal
and
postnatal
growth
retardation
•
Microcephaly
•
Microphthalmia
•
Malformed
ears
•
Micrognathia
or
retrognathia,
microstomia
•
Distinctively
clenched
fingers
(index
finger
overriding
the
middle
finger
and
the
fifth
finger
overriding
the
fourth
finger)
•
Other
congenital
malformations
including
congenital
heart
defects,
short
sternum,
and
rocker-bottom
feet
•
5%–10%
of
children
survive
beyond
the
first
year
•
Hypotonia
in
infancy,
hypertonia
in
older
children,
central
apnea
and
seizures
•
Significant
developmental
delays
are
always
present
ranging
from
a
marked
to
profound
degree
of
psychomotor
and
intellectual
disability
•
All
children
acquire
abilities
such
as
recognizing
their
family
and
smiling
appropriately
•
Older
children
often
can
walk
with
a
walker,
understand
words
and
phrases,
use
a
few
words
or
signs,
crawl,
follow
simple
commands,
recognize
and
interact
with
others
and
play
independently
•
Most
of
the
children
have
feeding
difficulties
that
often
require
tube
feeding
in
the
neonatal
period
or
placement
of
gastrostomy
in
older
children
•
Both
sucking
and
swallowing
problems
can
be
present
•
Gastroesophageal
reflux
is
common
and
often
severe
continues

124
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Trisomy
21
•
Brachycephaly
•
Upslanting
palpebral
fissures
•
Epicanthic
folds
•
Flat
facial
profile
and
nasal
bridge
•
Low
set,
small
folded
or
dysplastic
ears
•
Open
mouth
and
protruded
tongue
•
Short
neck
•
Short
broad
hands
•
About
one-half
of
the
children
have
congenital
heart
disease
•
Almost
all
individuals
have
cognitive
impairment,
although
the
range
is
wide
•
Most
have
mild
to
moderate
intellectually
disability
•
Increased
risk
for
disruptive
behavioral
disorders,
such
as
attention-deficit
hyperactivity
disorder,
conduct/oppositional
disorder
or
aggressive
behavior
•
Increased
risk
for
gastrointestinal
tract
anomalies
including
duodenal
atresia
or
stenosis,
esophageal
atresia
and
tracheoesophageal
fistula,
Hirschsprung
disease,
and
celiac
disease
•
Oral
motor
feeding
problems
including
oral
hypersensitivity
are
quite
common
•
Pharyngeal
phase
dysphagia
including
aspiration
can
occur
Angelman
syndrome
15q11.2
•
Microbrachycephaly,
pale
blue
eyes,
maxillary
hypoplasia,
,
deep-set
eyes,
large
mouth,
and
widely
spaced
teeth
•
Developmental
delay
evident
is
by
6–12
months
of
age,
sometimes
associated
with
truncal
hypotonia
•
Intellectual
disability
•
Severe
speech
impairment
•
Gait
ataxia
and/or
tremulousness
of
the
limbs,
puppet
gait
(arms
held
up
with
flexion
at
wrists
and
elbows)
•
Difficulty
with
breast•
or
bottle-feeding
including
problems
of
apparent
uncoordinated
sucking,
tongue
thrusting,
and
poor
breast
attachment
•
In
later
infancy,
gastroesophageal
reflux
can
occur
Table
3–6.
continued

125
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Angelman
syndrome
continued
•
A
unique
behavior
with
an
inappropriate
happy
demeanor
that
includes
frequent
laughing,
smiling,
and
excitability
•
Microcephaly
and
seizures
are
also
common
Rett
syndrome
(classic)
Xq28
Please
see
neurodevelopment
column
•
Loss
of
hand
skills
•
Loss
of
spoken
language
•
Gait
abnormality
and
stereotypic
hand
movements
•
A
mandatory
criterion
of
a
period
of
regression
followed
by
recovery
or
stabilization
•
Most
individuals
have
epilepsy
•
Undernutrition
and
poor
growth
are
common
•
Early
food
texture
intolerance
and
food
refusal,
low
use
of
self-feeding
•
Oropharyngeal
and
gastroesophageal
dysfunction
are
common
continues

126
Common
Genetic
Conditions
Cytogenetic
Location
Anomalies
Neurodevelopmental
Profile
Feeding
Abnormalities
Williams-Beuren
syndrome
7q11.23
•
Prenatal
growth
deficiency
•
Postnatal
failure
to
thrive
•
Cardiovascular
disease
(elastin
arteriopathy,
peripheral
pulmonary
stenosis,
supravalvar
aortic
stenosis,
hypertension)
•
Distinctive
facies
(broad
forehead,
bitemporal
narrowing,
periorbital
fullness,
a
stellate/
lacy
iris
pattern,
strabismus,
short
nose,
broad
nasal
tip,
malar
flattening,
long
philtrum)
•
Thick
vermilion
of
the
upper
and
lower
lips,
wide
mouth,
malocclusion,
small
jaw
•
Large
earlobes
•
Connective
tissue
abnormalities,
growth
abnormalities
•
Endocrine
abnormalities
(hypercalcemia,
hypercalciuria,
hypothyroidism,
and
early
puberty)
•
Harsh,
brassy,
or
hoarse
voice
•
Hypotonia
and
hyperextensible
joints
can
result
in
delayed
attainment
of
motor
milestones
•
Intellectual
disability
(usually
mild)
•
A
specific
cognitive
profile
(strengths
in
verbal
short-
term
memory
and
language
and
extreme
weakness
in
visuospatial
construction)
•
Unique
personality
characteristics
including
overfriendliness,
empathy,
generalized
anxiety,
specific
phobias,
and
attention
deficit
disorder
•
Disordered
suck
and
swallow,
textural
aversion,
and
vomiting
•
Prolonged
colic
(4
months)
may
be
related
to
gastroesophageal
reflux
•
Chronic
constipation
Table
3–6.
continued

Dysphagia in children with genetic
syndromes usually represents only one part
of a much broader spectrum of neuro
developmental dysfunction. Nonetheless,
dysphagia may be the most easily recog-
nized manifestation of these neurodevel-
opmental disorders because feeding is such
an important and pervasive daily activity.
For example, Prader-Willi syndrome can
be recognized initially by a history of poor
fetal movement in utero, significant hypo-
tonia, and dysphagia in the neonatal period.
Physical features are often subtle, particu-
larly in the 1st year of life, and include
prenatal or postnatal growth retardation,
narrow bifrontal diameter, almond-shaped
eyes, and hypogonadism (Figure 3–18).
Dysphagia is often severe in the neonatal
period, commonly requiring nasogastric
tube feeding; however, it is usually tran-
sient. Slowing of linear growth, small hands
and feet, obesity, global developmental
delays/intellectual disability, behavioral
problems, and hyperphagia are common
problems in the preschool and school-
age years. Prader-Willi syndrome can be
considered an autosomal dominant dis
order that is caused by deletion or disrup-
tion of a gene or several genes on the proxi-
mal long arm of the paternal chromosome
15 or maternal uniparental disomy 15, due
to the fact that gene(s) on the maternal
chromosome(s) 15 are virtually inactive
through imprinting.
Figure 3–18. A–B. A 1-year-old boy diagnosed with Prader-Willi syndrome. Narrow bifrontal
diameter and abnormal-shaped eyes can be appreciated.
A B

Clinical Approaches to
Identifying the Common
Etiologies of Dysphagia
in Childhood
A developmental and health history is essen-
tial in identifying acute, chronic static, and
chronic progressive neurologic and health
disorders resulting in abnormal swallowing
and feeding. Table 3–7 is a diagnostic sum-
mary of some of the more common etiolo-
gies associated with dysphagia. Conditions
producing dysphagia can be classified as
acute or chronic. Acute and chronic CNS
disorders are usually characterized by more
widespread neurologic dysfunction in addi-
tion to dysphagia. Table 3–1 illustrates that
both central and peripheral nervous system
diseases can be degenerative and therefore
progressive. In these disorders, previously
mastered developmental and feeding skills
are lost. A detailed feeding history, particu-
larly regarding the oral, pharyngeal, and
esophageal phases of swallowing, may be
suggestive of these disorders. More com-
mon pediatric, progressive CNS diseases
producing dysphagia include the Arnold-
Chiari malformation types 1 and 2, intra-
cranial tumors, and various leukodystro-
phies. Arnold-Chiari type II malformation
is a very frequent finding in children with
spina bifida and can result in static or pro-
gressive dysphagia (Hesz Wolraich, 1985).
Arnold-Chiari type I can be an isolated
finding in fewer than 1% of all children
(Yarbrough et al., 2011). The most com-
mon presentation, particularly in infants, is
subacute or acute oropharyngeal swallow-
ing problems or gastroesophageal reflux.
Headache as an initial symptom is more
common in children during the preschool
years (Albert, Menezes, Hansen, Greenlee,
Weinstein, 2010). Leukodystrophies are
disorders marked by degeneration of the
white matter of the brain and characterized
by demyelination and glial reaction. These
and other disorders that affect the mental
status, upper extremity function, or posture
can seriously impair the acquisition and
delivery of food.
Cardiopulmonary Disorders
and Dysphagia
Various cardiopulmonary disorders can
significantly compromise respiration dur-
ing oral feedings. Chronic lung disease and
congestive heart failure are commonly man-
ifested by progressive tachypnea or fatigue
during oral feedings. Infants with congenital
heart disease are at higher risk for feeding
problems due to a number of factors includ-
ing longer duration of respiratory support,
use of bypass, and neurological complica-
tions (Jadcheria, Vijayapal, Leuthner,
2009; Pillo-Blocka, Adatia, Sharieff, Mc-
Crindle, Zlotkin, 2004). Infants with cya-
notic heart disease, and those with hypo-
plastic left heart syndrome have increased
prevalence of feeding and growth problems
(Davis et al., 2008; Jadcheria et al., 2009;
McGrattan et al., 2017; Skinner et al., 2005).
Gastroesophageal Disease
and Dysphagia
Gastroesophageal disease can also be mani-
fested as progressive dysphagia. Worsening
of gastroesophageal reflux disease, esopha-
gitis, or the development of esophageal
strictures are not uncommon in children
with severe CP and dysphagia (Sullivan,
2008). The more recently recognized extra-
esophageal reflux disease (EERD) can have
direct untoward effects on the pharynx and
larynx, making swallowing difficult. This
topic is discussed in further detail in Chap-
ters 4 and 5.

129
Table
3–7.
Etiologies
of
Dysphagia
in
Childhood
Site
of
Pathology
Acute
Chronic
Static
Progressive
Central
nervous
system
Hypoxic-ischemic
encephalopathy
Cerebral
infarctions
Intracranial
hemorrhage
Infections
•
Meningitis
•
Encephalitis
•
Poliomyelitis
•
Botulism
•
Syphilis
Acute
bilirubin
encephalopathy
Metabolic
encephalopathies
•
Aminoacidopathies
•
Disorders
of
carbohydrate
metabolism
Neonatal
withdrawal
syndrome
(heroin,
cocaine,
barbiturates)
Traumatic
encephalopathies
and
brain
stem
injuries
Arnold-Chiari
malformation
(types
1
and
2)
Genetic
syndromes
Familial
dysautonomia
(Riley
Day)
Mobius
sequence
Congenital
anomalies
of
the
brain
Cerebral
palsy
Neurodevelopmental
disorders
(e.g.,
Rett
syndrome,
autism,
intellectual
disabilities)
Chronic
postkernicteric
bilirubin
encephalopathy
Arnold-Chiari
malformation
(types
1
and
2)
Syringobulbia
Intracranial
malignancies
•
Tumors
•
Leukemia
•
Lymphoma
Degenerative
white
and
gray
matter
diseases
•
Lysosomal
storage
diseases
(e.g.,
mucopolysaccharidoses,
Niemann-
Pick,
Krabbe’s,
metachromatic
leukodystrophy,
Tay-Sachs,
nephropathic
cystinosis)
•
Mitochondrial
disorders
(e.g.,
Leigh
disease,
carnitine
deficiency)
•
Peroxisomal
disorders
(e.g.,
Zellweger’s,
adrenoleukodystrophy)
•
Purine
and
pyrimidine
disorders
(e.g.,
Lesch-Nyhan)
Disorders
of
copper
metabolism
(e.g.,
Wilson’s
disease,
Menkes’
disease)
continues

130
Site
of
Pathology
Acute
Chronic
Static
Progressive
Central
nervous
system
continued
DNA
repair
deficiency
syndromes
(e.g.,
ataxia
telangiectasia,
Cockayne
syndrome)
Neurodegeneration
with
brain
iron
accumulation
disorders
Infections
(e.g.,
HIV
encephalopathy)
Spinocerebellar
disorders
Dystonia
musculorum
deformans
Multiple
sclerosis
Amyotrophic
lateral
sclerosis
Anterior
horn
cell
Infantile
spinal
muscular
atrophy
Peripheral
nervous
system
Acute
inflammatory
polyradiculoneuropathy
Polyneuropathies
Polyneuropathies
Neuromuscular
junction
Hypermagnesemia
Myasthenia
gravis
Table
3–7.
continued

131
Site
of
Pathology
Acute
Chronic
Static
Progressive
Muscles
Polymyositis
Dermatomyositis
Congenital
myopathies
(e.g
Nemaline
Rod)
Myotonic
dystrophy
Congenital
muscular
dystrophy
Infantile
faciocapulo-humeral
dystrophy
Metabolic
myopathies
(e.g.,
glycogen
storage
disease)
Duchenne’s
muscular
dystrophy
Respiratory
tract
Otis
media
Sinusitis
Severe
chronic
lung
disease
and
airway
obstruction
(e.g.,
bronchopulmonary
dysplasia)
Cardiovascular
cyanotic
Congenital
heart
disease
Congenital
heart
disease
disorders
Gastrointestinal
tract
Dental
caries/abscesses,
gingivitis
Gastroesophageal
reflux,
dental
caries/
abscesses,
gingivitis
Gastroesophageal
reflux
and
esophagitis,
esophageal
stricture
Dental
caries/abscesses,
gingivitis
Psychological
Disorders
of
caregiver-child
interaction

A precise swallowing and feeding his-
tory is useful and important. Changes in
oral feeding techniques can lead to new
feeding problems in children with preex-
isting dysphagia. Swallowing efficiency is
often dependent on food texture. Newly in-
troduced foods with more than one consis-
tency can result in increased coughing and
gagging, particularly in children with neu-
rogenic dysphagia. Changes in the delivery
of oral feedings also can lead to problems.
A persistently strong tongue protrusion
reflex is commonly observed in children
with CP and dysphagia. This reflex alone
rarely interferes with bottle-feedings but
can result in excessive oral loss of semisolids
when presented on a spoon. These examples
illustrate potential new feeding problems
in children with nonprogressive dysphagia.
The astute clinician should note, however,
that these developments do not result in the
loss of previously mastered feeding skills.
Generally, the majority of conditions listed
in Table 3–7 can be suspected by history
and then confirmed by a careful physical
examination.
Case Studies
The cases presented in this section empha-
size the challenges of diagnosis and manage-
ment of children with complex swallowing
and feeding disorders.
Case Study 1
RR was a 4½-year-old boy who initially pre-
sented to an outpatient feeding clinic with
his mother expressing concerns that “he
doesn’t eat” and that this had been a prob-
lem since he was 2 years of age. He refused
much of the meals his mother prepared for
him. He had a history back to 21 months of
age with intermittent vomiting while tak-
ing omeprazole. He was being treated for
constipation as well. Mother also reported
that he seemed “sad” most of the time since
his father fled to Mexico 9 months prior to
presentation to clinic.
Past history was notable for full-term
birth following an uncomplicated preg-
nancy. He was delivered by uncomplicated
spontaneous vaginal delivery. Birth weight
was 8 pounds. He did well and was dis-
charged in 2 days. Mother breastfed him and
provided formula until he reached 8 months
of age. He was described as a good feeder
and took spoon-feedings readily. There was
no history of vomiting at that time. He was
hospitalized for a respiratory syncytial viral
infection at 4 months of age. Emesis and
poor appetite commenced at 21 months,
and he was hospitalized at 28 months of age
for failure to thrive. An upper gastrointes-
tinal study and small bowel follow-through
were normal. VFSS was normal. An esopha-
gogastroduodenoscopy (EGD) and antrum
biopsy revealed chronic active gastritis and
abundant Helicobacter-type organisms. He
was given a course of antibiotics (amoxacil-
lin and clarithromycin), and omeprazole,
and started on a high-calorie formula.
Subsequently, his mother reported that
the vomiting and intermittent epigastric
pain continued. Follow-up EGD at about
4 ½ years of age revealed resolved gastritis.
Chemistry panel, complete blood count,
celiac panel, and thyroid panel were normal,
and fecal calprotectin was elevated, which
indicated probable intestinal inflammation.
Physical examination at 4½ years in-
cluded Wt = 12.3 kg (Z score = −3.23), Ht
= 93.3 cm (Z = −2.75), and HC = 50 cm
(−0.47). The patient was described as a
“reserved, serious, temperamental child”

who stayed close to his mother. He was non-
verbal during the visit, and the neurologi-
cal examination was normal. There was no
evidence of oral sensorimotor delays or dys-
function. High-calorie supplements contin-
ued. Psychology was consulted concerning
food refusal and because RR appeared “sad”.
Two weeks later he was admitted again
for undernutrition (FFT) and started on
nasogastric (NG) tube feedings. Follow-
up VFSS revealed some vallecular pooling
after swallowing but no airway penetration
or aspiration.
Over the ensuing year, he had fluctuating
weight gain, with intermittent use of the NG
tube for feeding. He continued with omepra-
zole and was treated for constipation. Clini-
cians felt that there was a large “behavioral
component” to his food refusals. More
efforts were made to obtain mental health
consultation for family and child stress.
At 6½ years of age, RR was referred by
his pediatrician to see a physical therapist
for evaluation of persistent “neck pain over
the past year.” Mother reported that his neck
pain seemed to have followed a car accident
in which the family car was rear-ended.
Mother reported that he typically woke up
in the morning with the neck pain. The
neck pain got worse during the day when
he would turn his head suddenly. When his
neck pain was severe, he would also vomit.
At times, his neck pain seemed to move up
into his head and he would get “sweaty.” The
physical therapist’s examination revealed
that RR seemed withdrawn, and he had a
right torticollis, and scoliosis. The therapist
recommended an x-ray of his neck (which
was never obtained) and started therapy
once per week.
RR was seen again in feeding clinic
about 1 month later. At that time, mother
reported fluctuating appetite. He had food
refusal and temper tantrums when meals
were offered to him. Neck pain associ-
ated with projectile emesis was noted, and
mother also stated that during periods of his
neck pain, he would get “dizzy” and often
grabbed pieces of furniture to stop from
falling. Physical examination revealed a
“cachectic, low energy, depressed child.” He
was noted to have a right head tilt. Neuro-
logical examination was otherwise normal.
Hospital admission was recommended.
During his hospitalization, a cranial
MRI with and without contrast revealed a
4.5 × 4.5 cm fourth ventricle posterior exo-
phytic mass from the dorsal aspect of the
brain stem. The mass resulted in obstructive
hydrocephalus and severe foramen mag-
num stenosis with associated upper cord
edema (Figure 3–19). Subsequent surgical
pathology was consistent with a pilocytic
astrocytoma. He underwent a suboccipital
craniotomy with resection of the posterior
fossa dorsal exophytic brain stem tumor.
The tumor was found to have infiltrated
into the medulla and peduncle tissue. The
tumor was dissected off the surrounding
cerebellar structures, and the tumor was
widely debulked in a superior, posterior,
and lateral fashion resulting in a subtotal
resection (residual infiltrating brainstem).
The postoperative course was uncompli-
cated and he was discharged to home after
12 days.
Postoperative cranial MRI revealed
interval resection of a majority of the previ-
ously seen dorsal medulla enhancing mass.
Persistent area of abnormal T2 hyperinten-
sity was noted in the dorsal medulla and
dorsal cervicomedullary junction. Subse-
quent follow-up revealed improved appe-
tite, weight gain, and resolution of neck
pain and emesis. His coordination and
hand strength improved as well. Subsequent
appointments revealed normal appetite and
food acceptance.

Comment
RR emphasizes the challenges in diagnos-
ing chronic progressive conditions that
result in dysphagia or feeding problems in
childhood. RR’s presentation at 28 months
of age with a 7-month history of emesis,
food refusal, weight loss, and emaciation, is
quite atypical for gastroesophageal reflux,
food refusal, or dysphagia. Generally gas-
troesophageal reflux will present in the first
year of life with emesis, food refusal, and
slowing of weight gain. The documenta-
tion of chronic active gastritis, and Helico-
bacter infection at the time was a legitimate
explanation for his clinical course. How-
ever, on subsequent follow-up, there was no
improvement of his emesis and food refusal
with appropriate treatment even though
follow-up EGD demonstrated resolution of
his gastritis. These problems also occurred
in the context of significant family stress,
noted by RR’s presentation as a withdrawn
and somewhat depressed child.
On subsequent follow-up, RR had new
onset of neck/occipital pain that was worse
on arising in the morning. The duration of
the headaches extended over 1 year, seemed
to progressively worsen, became incapaci-
tating, and were associated with dizziness
and vomiting. Eventually he developed a
head tilt and “scoliosis,” and neck pain wors-
ened with rapid turning of his head.
Ghodsi and colleagues (2013) retro-
spectively described the clinical courses of
11 children, age range 11 months to 7 years
with confirmed exophytic gliomas of the
medulla. These brain tumors grow very
insidiously and result in a long history of
slowly progressive symptoms. Children typ-
ically present with unexplained vomiting,
failure to thrive, and new-onset swallowing
problems. Headaches usually occurred in
older children. Honig and Charney (1982)
in a classic review of headaches associated
with brain tumors, described common
characteristics including recurrent morning
headaches, headaches that would awaken
Figure 3–19. A–B. Cranial MRI with and without contrast revealed a 4.5 × 4.5 cm fourth ventricle
posterior exophytic mass from the dorsal aspect of the brain stem.The mass resulted in obstruc-
tive hydrocephalus and severe foramen magnum stenosis with associated upper cord edema.
A B

children in the evening and were intense,
prolonged, and incapacitating. Generally
headaches were progressive in quality, fre-
quency, and pattern. Recent onset or in-
creased frequency of emesis was also charac-
teristic. Common physical finding included
diplopia, papilledema, and head tilt.
Summary
It is impossible to know when RR became
symptomatic from his posterior fossa tumor.
Certainly, the presentation of unexplained
vomiting and weight loss later in infancy is
somewhat atypical, but Helicobacter infec-
tion was noted and treated. However, the
persistence of vomiting despite a normal
upper GI study and small bowel follow-
through, multiple formula changes, and
prolonged use of a proton pump inhibitor
and persistent undernutrition (FTT) and
cachexia should result in careful review of
the differential diagnosis of any child. The
new onset of occipital headaches, particu-
larly on arising in the morning, and head tilt
led to the diagnosis. Continued follow-up of
patients, documentation of progression of
symptoms, and a high index of suspension
should lead to early and accurate diagnosis.
Case Study 2
Relevant History
EA is a nearly 5-year-old female with prena-
tal diagnoses of initial intrauterine growth
restriction (IUGR) and cleft lip and pal-
ate. She was later diagnosed with Wolff-
Hirschhorn syndrome associated with
bilateral cleft lip and palate (unrepaired
cleft palate), hearing loss, glaucoma, sei-
zures, developmental delays, C1-C2 ver-
tebral instability, chronic kidney disease
(hypoplastic kidneys), and neurogenic dys-
phagia. A cervical collar was prescribed. She
presented for a clinical feeding evaluation
and VFSS as part of her follow-up care with
an interdisciplinary feeding team.
Previous neuroimaging revealed atlanto-
axial instability consisting of an abnormal
cervical vertebrae-1 arch with the right
arch indenting the spinal cord, and flexion
extension x-ray revealing 1 cm of atlanto-
occipital motion, and low-lying spinal
conus at the level of the L2-L3 disc space
suggestive of a tethered spinal cord.
Her early care was provided at an out-
side facility in another state. She under-
went a bilateral cleft lip repair at 6 months
of age, at which time a permanent (non-
removable) hard plastic palatal obturator
was placed and secured into palatal shelves
with pins. She had consistently been noted
to have hypotonia, global developmental
delays, and feeding problems. The obtura-
tor appeared to have “shifted” in her mouth.
Feeding History
Parent reported that EA has grown ade-
quately although numerous recommenda-
tions for gastrostomy tube placement had
been made over the years. EA continued to
be fed totally orally with a diet of pureed
foods, including blended family meals and
meats. She coughed and choked when given
thin liquids (water, juice) via a spout cup.
She previously consumed 8 oz (240 ml)
Pediasure each morning for additional
nutrition supplementation, but began refus-
ing a few months prior to presentation to
the Feeding Team. Her mother provided
high-calorie pureed foods, such as oat-
meal supplemented with coconut oil, flax
seed, chia seeds, and DuoCal. EA did not
self-feed, but would accept a spoon with a
very small amount of puree (approximately
1/3 teaspoon) during four small meals per
day. Her mother denied signs/symptoms of

aspiration with pureed textures. No dry
crunchy textures were offered due to EA’s
impaired oral motor skills. Feeding observa-
tion revealed that EA held food in her mouth
forprolongedtimeperiod.Shedemonstrated
difficulty with bolus manipulation that re-
sulted in spillage of food from her mouth.
Developmental History and Status
EA was not using any words, hands in her
mouth most of the time, she was rolling both
ways but not sitting, reaching, or grasping.
After transfer of care at 2 ½ years of age, EA’s
growth was noted to slow considerably. At
nearly 5 years of age, weight was 8.8 kg (19.4
pounds), Z-score of −8.03. Her body mass
index was recorded at 0.01% and Z-score
of −4.49. Head circumference was 42.5 cm
(severe microcephaly). Growth parameters
were consistent with chronic malnutrition.
Physical Examination
Notable physical findings included severe
microcephaly, broad nasal bridge, high fore-
head (“Greek helmet” facies), cervical collar
in place, repaired cleft lip, cleft of hard and
soft palate with prosthesis in place, tapering
fingers, tuft of hair and round large sacral
pit over lumbosacral area (Figure 3–20).
Neurological examination was abnormal.
Findings included lack of visual tracking of
red ring or light, moderate truncal hypo-
tonia, prominent truncal and leg extensor
arching and tone, and spasticity of lower
extremities.
Instrumental Swallow Study
During her VFSS, EA demonstrated delayed
oral transit skills with decreased propulsive
forces that resulted in significant residue
along the tongue base. EA produced an
average of three to four swallows to clear
each bolus from the pharynx, which indi-
cated significant effort to clear very small
bites of puree. During a trial with a bite-
sized piece of banana, no tongue lateraliza-
tion was noted. She did not chew the food,
but just swallowed the small banana piece
whole, which resulted in gagging. With very
small sips of thin liquid, she initiated sev-
eral swallows with delay due to oral hold-
ing, and one episode of microaspiration
with no cough was observed near the end
of the study due to delayed initiation of the
pharyngeal swallow.
Team Recommendations
for Management
Recommendations included gastrostomy
tube placement due to chronic malnutri-
tion with strategies to increase caloric
intake during oral feeds. Neurosurgery
Figure 3–20. 5-year-old with microcephaly,
broad nasal bridge, high forehead (“Greek
helmet” facies), cervical collar in place.

plans included occipital-C2 posterior spi-
nal fusion and eventual repair of tethered
spinal cord.
Comment
This child illustrates many of the important
reasons why interdisciplinary care is critical
in the management of swallowing and feed-
ing problems, particularly in children with
structural as well as functional anomalies of
early development.
At birth, EA was noted to have IUGR
and cleft lip/palate. IUGR alone has been
associated with higher risk for poorer neu-
rodevelopmental outcomes. Specifically,
term infants with IUGR are at higher risk for
CP, lower intelligence, poor academic perfor-
mance, low social competence, and behav-
ioral problems (Walker Marlow, 2008).
EAwasdiagnosedwithWolf-Hirschhorn
syndrome (WHS) based on the presence of
severe IUGR, microcephaly, “Greek hel-
met” facies, and closure defects (cleft lip
or palate, coloboma of the eye, and cardiac
septal defects). WHS is a contiguous gene
syndrome caused by partial loss of mate-
rial from the distal portion of the short
arm of chromosome 4 (4p16.3). Over 50%
of children with WHS have required feed-
ing gastrostomy tubes in order to maintain
their nutritional status (Battaglia, Filippi,
Carey, 2008).
This child also illustrates the complex
relationship between “structure” versus
“function” in pediatric dysphagia. It has
been generally accepted that cleft lip and
palate can result in swallowing and feeding
difficulties due to an anatomical difference
(i.e., unrepaired cleft palate) secondary to
an abnormal mechanical function. EA was
not a candidate for primary cleft palate
repair. Thus, a permanent obturator had
been placed when EA was an infant as a
prosthetic aid designed to obturate the cleft
and restore the separation between the oral
and nasal cavities to improve feeding func-
tion. However, it was not completely suc-
cessful (not surprising given reports in the
literature, e.g., Glenny, Hooper, Shaw, Reilly,
Kasem, Reid, 2004; Prahl, Kuijpers-Jagt-
man, Van’t Hof, Prahl-Anderson, 2005).
Growth was significantly impacted, nasal
regurgitation continued to occur with oral
feedings, and EA experienced difficulties
with generating appropriate swallowing
pressures for bolus propulsion and pharyn-
geal clearance.
It must be kept in mind that EA’s oro-
pharyngeal dysphagia occurred in the con-
text of global developmental delays and
abnormalities on neurological examination.
As previously noted, in high-risk infants,
the presence of hypotonia is an indepen-
dent predictor for feeding problems (Crap-
nell et al., 2013; Zehetgruber et al., 2014). In
our experience, oral sensorimotor function
in young children is consistent with over-
all gross motor functioning and cognitive
status. In this context, since EA’s general
motor and cognitive skills were less than
a 6-month level, it was not surprising that
she had not yet developed vertical chewing
or tongue lateralization. All of these factors
resulted in significant effort for swallowing
small amounts of food and liquid, leading
to fatigue during meals and chronic malnu-
trition, with weight loss occuring after she
began refusing her nutritional supplement.
Case Study 3
History
“Timothy” presented at 12 years of age, with
primary diagnosis of Trisomy 21 (Down
syndrome). He was admitted to a children’s
hospital because of a 2-month history of
persistent bilateral lower lobe pneumonia.

Although he had received a number of
courses of antibiotics, he had not improved
clinically. His mother stated that during the
previous 6 months, he had increasing dif-
ficulty in swallowing solid food, had poorer
speech, and had begun drooling excessively.
His past medical history was significant for
C1–C2 vertebral instability that had led to a
posterior cervical spinal fusion. He had pre-
viously been diagnosed with a neurogenic
spastic bladder. He was on no medications
at the time.
and Imaging Findings
“Timothy” was an alert, cooperative pre-
teenage boy in no acute distress. Inspiratory
rales were noted over both lower lung fields.
He drooled excessively, and his speech
reflected flaccid dysarthria. Jaw clonus was
observed, and no gag reflex was elicited. All
four extremities were weak, his arms more
so than his legs. Deep tendon reflexes were
increased in the legs. Plantar responses were
up-going. Gait was mildly ataxic; mild dys-
metria was noted. Romberg response was
observed, and cremasteric reflex was absent.
Recent chest x-rays revealed chronic
bibasilar infiltrates. A VFSS showed aspi-
ration of liquid during swallows. A cranial
magnetic resonance image revealed marked
C1–occiput instability. Herniation of the
cerebellar tonsils was noted. Additionally,
the clivus was compressing the anterior
medulla (Figure 3–21).
Surgical Procedure and Follow-Up
Timothy underwent an occiput to C1–C2
fusion and halo vest application. Oral feed-
ings were discontinued, and a feeding gas-
trostomy tube was placed. His pneumonia
subsequently cleared.
Figure 3–21. A cranial magnetic resonance image (midline sagittal view)
revealed herniation of the cerebellar tonsils. The clivus (arrow) is shown to
be compressing the medulla anteriorly.

Timothy’s neurologic status and swallow-
ing gradually improved over the following
year. One year after discharge, his gait was
normal. A follow-up VFSS revealed mild
pharyngeal dysmotility but no aspiration.
Oral feedings were gradually resumed with-
outdifficulty.Gastrostomytubewasremoved
by the end of the year following surgery.
Comment
This case emphasizes the diagnostic impor-
tance of recognizing progressive dysphagia,
even in children with recognized genetic
syndromes. Knowledge of the potential CNS
complications of C1–C2 vertebral instabil-
ity in children with Down syndrome and a
detailed feeding history were essential in his
diagnosis. The compression of his medulla
by the floor of his skull directly resulted in
his dysphagia and chronic aspiration.
Timothy’s follow-up care was maxi-
mized by the interdisciplinary team. VFSS
in conjunction with clinical oral sensori
motor feeding assessments were essential
for choosing safe and effective methods for
his feeding.
Case Study 4
History at Presentation
to Feeding Team
PC presented to a Swallowing and Feeding
clinic at 2 months of age with a history of
“slow to feed.” She was taking 4 ounces for-
mula at each feeding by bottle/nipple over
1 hour, with some coughing and oral loss.
Mother had attempted to breastfeed for the
first 2 weeks of life, but switched to bottle-
feeding. Nurse midwife questioned the
presence of a “tight lip.” Frequent emesis
was treated with Ranitidine, a histamine-2
blocker.
Pregnancy was complicated by pre-
eclampsia and severe migraine headaches.
Scheduled cesarean section occurred at
term. Birth weight was 7 pounds, 3 ounces,
and neonatal period was uncomplicated.
Clinic feeding observation revealed a
munch pattern with excessive jaw excursion
noted while she was sucking. She appeared
to have minimal intraoral sucking pressure.
She had minimal resistance when the nipple
was eased out of her mouth. Sweating was
noted on her forehead, and she seemed to
fatigue within 6 minutes into the feed. Phys-
ical examination revealed normal growth
parameters and a head circumference of
40 cm (70%). There were no distinguish-
ing physical features. Upper and lower lip
ties were thick and somewhat restrictive.
Ankyloglossia was suspected. The infant
visually tracked a red ring horizontally and
vertically. The neurological examination
was normal except that the infant seemed
quite restless, rotating back and forth from
side to side.
Follow-Up From Initial
Feeding Evaluation
She was referred for a frenulectomy at
4 months of age. Parents reported that the
surgery did not alter her feeding skills. She
continued to “munch” on the nipple and
frequently choked and coughed during
oral feedings. At 8 months of age a VFSS
revealed inconsistent mild to moderate
delayed initiation of swallow (especially
when she seemed tired). There were no
signs of laryngeal penetration or aspira-
tion with thin liquids and pureeds. She had
no pharyngonasal backflow or pharyngeal
residue. At 8 months, PC had not started to
roll consistently. She was cooing, and occa-
sionally would laugh out loud. There was
no razzing or babbling. She was not reach-
ing for toys. On physical examination, she

was “chubby” appearing. Weight-for-length
ratio was over the 97%ile. Head circumfer-
ence was at the 90%ile. On neurological
examination, she was quite hypotonic with
axillary slip through. Muscle strength was
within normal limits. Deep tendon reflexes
were brisk. She was fisted at least 75% of the
time. She seemed to be in constant motion,
and often slapped the back of her right fist
against her mouth repetitively. She seemed
overly fixated as she repetitively looked at
her hands, but there were no hand wring-
ing movements. There was truncal exten-
sor arching in supported standing, but she
did not bear weight on her legs. Eye con-
tact with the examiner was fleeting at best.
She did not reach for objects. Cranial MRI
was normal. Chemistry panel, T4, and TSH
were normal. She was referred to the Genet-
ics service for evaluation of possible atypical
Rett syndrome.
Genetics Evaluation
PC was seen by a geneticist at 10 months
of age. Notable physical findings included a
symmetrical but “wide” face, and deep-set
eyes, tapering fingers, and diffuse hypoto-
nia. No definite syndrome could be iden-
tified, and a chromosome microarray was
ordered. The microarray revealed a 4.8 Mb
deletion at 15q11.2q13.1 that spanned the
type II Prader-Willi syndrome/Angelman
syndrome (PWS/AS) region. This is the
smaller of the two types of deletions. Meth-
ylation analysis was consistent with a diag-
nosis of Angelman syndrome.
Neurologic Sequelae
At 11 months of age, she had her first sei-
zure, lasting over 10 minutes, and was
started on zonisamide and levetiracetam.
A follow-up assessment at 13 months of
age revealed language and cognitive skills
at a 2- to 3-month level and motor skills at
a 3- to 4-month level. An ophthalmology
examination revealed hypopigmented fundi
(known association with Angelman syn-
drome), and high astigmatism in both eyes,
in the amblyogenic range, and glasses were
prescribed.
At 18 months of age, PC was admitted
to the pediatric intensive care unit for status
epilepticus. Video EEG showed status epi-
lepticus, with left greater than right electro-
clinical seizures, characterized by rhythmic
jerking of her right hemibody. Her MRI
showed extensive white matter edema, left
more than right, thought to be post-ictal.
She was treated with benzodiazeoines, fos-
phenytoin, and lacosamide. Eventually her
seizures resolved, and she was discharged
home on zonisamide, clorazepate, and
lacosamide. Because of feeding difficulties,
she was sent home with an NG tube.
Feeding Follow-Up
A follow-up VFSS was completed at 18
months of age with thickened liquids pre-
sented via Dr. Brown bottle with Stage 3
nipple. The patient tolerated the exam
well. Oral bolus control and preparation
were moderately reduced with limited milk
transfer during active nutritive sucking
bursts. PC demonstrated excellent initiation
of latch on a nipple. She had difficulty sepa-
rating from her bottle consistent with par-
ent report that she sucks on it “all the time.”
With honey-thick consistency PC took
minimal volumes. Initiation of the pharyn-
geal phase was severely delayed with spillage
to the valleculae and pyriform sinuses with
no noticeable difference between consisten-
cies. Once initiated, laryngeal elevation was
typically delayed but complete resulting in
delayed but complete epiglottic deflection.
Velopharyngeal closure was normal. Tongue
base to pharyngeal wall contact was moder-

ately reduced. For nectar-thick liquids there
was noted to be frank aspiration from the
pyriform sinuses prior to swallow initiation.
She responded with an immediate cough.
At 23 months of age, she was making
guttural sounds, no cooing, but she laughed
out loud. PC was not turning to her moth-
er’s voice. She was not rolling. She was pre-
dominantly moving her left hand and arm
but did not reach or grasp objects. She made
inconsistent responses to parent’s voice. On
physical examination, weight was at 48%,
length was at 35%, and head circumference
was 49 cm (87%). Figure 3–22A highlights
some key facial features including wide face,
deep set eyes, wide mouth, tongue protru-
sion, and flexor posture of her arms, fisted
hands, and NG tube in place. Figure 3–22B
reveals wide spacing of her teeth. Neurolog-
ical examination revealed visual tracking of
red ring in left visual field. Some eye gaze
was noted to examiner’s face. Possible social
smile was inferred? Or noted? She made
some effort to bat at a ring with her left
hand. Arms flexed, R L, right hand was
more tightly fisted than left. Legs extended,
on vertical suspension. Consistent clasp
knife hypertonia was observed in her right
arm and leg. Observation of left arm and
leg revealed increased variable hypertonia
in flexion of left arm and extensor tone
of the left leg. Mild head lag was noted on
pull to sit. The team recommended a feed-
ing gastrostomy tube when PC was medi-
cally stable.
Comment
In review, Angelman syndrome is a neu-
rodevelopmental disorder characterized
Figure 3–22. A. PC’s facial features including wide face, deep set eyes, wide mouth, tongue
protrusion, and flexor posture of her arms, fisted hands, and nasogastric tube in place. B. Oral
cavity view highlights widely spaced teeth.
A B

by intellectual disability, abnormalities of
movement, typical abnormal behaviors, and
severe speech and language impairment.
Most occurrences are caused by the absence
of a maternal contribution to the imprinted
region on chromosome 15q11-q13. Prader-
Willi syndrome (PWS; 176270) is a clinically
distinct disorder resulting from paternal
deletion of the same 15q11-q13 region.
Typically, most children have at least
eight of the major characteristics of the syn-
drome including bursts of laughter, happy
disposition, hyperactivity, micro- and
brachycephaly, macrostomia, tongue pro-
trusion, prognathism, widely spaced teeth,
puppet-like movements, wide-based gait,
and intellectual disability and absence of
speech. Most patients (80.8%) have epileptic
seizures, starting after the age of 10 months.
In children under the age of 2 years, bursts
of laughter are found in 42.8% and mac-
rostomia in only 13.3%, but protruding
tongue is a constant feature (Buntinx et al.,
1995). Fryburg, Breg, and Lindgren (1991)
decribed the clinical presentation of young
infants with Angelman syndrome. All four
had choroidal pigment hypoplasia, severe to
profound global developmental delay, and
microcephaly of postnatal onset, seizures,
hypotonia, hyperreflexia, and hyperkinesis.
Retrospectively, by 1 year of age, PC
had many of the features of Angelman syn-
drome. PC had feeding problems dating
from the neonatal period, characterized
by abnormalities of the oral and pharyn-
geal phases of swallowing. Developmen-
tal delays were evident early on including
hypotonia with normal muscle strength,
motor delays, and significant delays in pre-
linguistic language development and visual
problem-solving. Early in the first year, PC
was described as very active. She had repeti-
tive behavior including rolling from side to
side, and visual fixation on hands. Physical
features in the first year were quite subtle
including deep-set eyes, and wide face. At
around 2 years of age, PC had more obvious
physical features.
One of the major differential diagnoses
to be considered in young infants who pres-
ent with significant hypotonia and feeding
problems has traditionally been Prader-
Willi syndrome. Similarly, in light of the
absence of significant physical features in
the age range, Angelman syndrome should
also be considered.
This case emphasizes the value of not
only detailed feeding assessments, but also
high-quality physical and neurodevelop-
mental examinations over time. Children
with unexplained global developmental
delays associated with feeding problems
should not only be referred to early inter-
vention and enrolled in feeding therapy, but
also have extensive evaluations to determine
the etiology of the feeding and developmen-
tal impairments. Finally, children like PC
are best cared for in environments in which
a broad range of pediatric specialists are
available who can work together as a coor-
dinated team.
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149
4The Upper Airway
and Swallowing
Robert Chun and Margaret L. Skinner
Summary
The intimate anatomic relationship of the
upper airway to the upper digestive tract
structures involved in swallowing neces-
sitates precise coordination of breathing,
laryngeal closure, upper esophageal open-
ing, and swallowing. Maintaining an effec-
tive and efficient airway to provide adequate
ventilation and prevent aspiration is an
important concern in infants and children
who present with swallowing and feeding
problems. Problems of dysphagia can be
secondary to both upper and lower airway
problems. Sequelae of dysphagia may also
contribute to airway disease.
The evaluation of the pediatric airway
requires a thorough knowledge of the rel-
evant upper airway anatomy, particularly
the larynx (see Chapter 2). Clinical exami-
nation of the child is often supplemented
with appropriate diagnostic studies, espe-
cially direct visualization of the larynx, tra-
chea, and esophagus. This chapter focuses
on the diagnosis and treatment of airway
problems, which are associated with swal-
lowing difficulties in children with specific
airway abnormalities, craniofacial anoma-
lies, and tracheostomy. Clinical case studies
are used to highlight salient points.
Introduction
Establishing and maintaining an adequate
airway are the first and most important
physiologic functions for the newborn
infant. After airway maintenance, the inges-
tion and digestion of adequate nutrients
are necessary to provide energy for optimal
growth and development. The ability of
humans to communicate using voice and
spoken language is made possible by the
development and adaptation of the upper
aerodigestive tract to support breathing and
swallowing and vocalizing functions (Lait-
man Reidenberg, 1993).
Swallowing and feeding problems may
first present with airway signs and symp-
toms. Conversely, an airway problem may
lead to difficulty with swallowing and feed-
ing. The occurrence of airway and feed-
ing problems is increased when head and
neck structural abnormalities, neurologic
impairment, inflammatory or infectious
disease, cardiac disease or metabolic dis-
orders are present. Swallowing problems
presenting initially as airway symptoms,
may occur in children with structural or
functional airway problems or gastrointes-
tinal (GI) disease, most commonly gastro-
esophageal/extra-esophageal reflux disease

(GERD/EERD) and eosinophilic esophagi-
tis (EoE).
An effective and safe airway is essential
to life. Recognition of a compromised air-
way during swallowing and feeding is essen-
tial when establishing an accurate diagno-
sis and optimal treatment plan. Airway
distress may present as increased work of
breathing, retraction of the sternal notch or
costovertebral angle, increased respiratory
rate, stridor, apnea or cyanosis. Further-
more, an evaluation of a child with a feed-
ing or swallowing difficulty associated with
airway distress must also include evaluation
of the cardiac, GI tract, and neurologic sys-
tems, as well as the ear, nose, throat, head,
and neck.
Clinical Evaluation
History
The clinical evaluation always begins with
a detailed history. Prenatal, perinatal, and
postnatal events may reveal prenatal infec-
tions (e.g., cytomegalovirus), intrauterine
toxin exposures (e.g., medications, alcohol,
or drugs), precipitous or traumatic deliv-
ery (e.g., vocal fold paralysis from difficult
vaginal delivery), or perinatal or postnatal
trauma (e.g., perinatal asphyxia from fetal
distress or intubation for meconium aspi-
ration). Polyhydramnios, which is exces-
sive accumulation of amniotic fluid, during
pregnancy may alert the clinician to func-
tional (usually neurologic) or structural
laryngeal/esophageal abnormalities, both
of which are associated with airway prob-
lems. Family history should be explored for
the presence of relatives with cleft lip and/
or palate, other craniofacial or congenital
anomalies (see Chapter 12), GERD (see
Chapter 5), syndromes and neurodevelop-
mental abnormalities (see Chapter 3).
In the older child, past medical history
might reveal severe neonatal GERD, which
can herald the development of EERD later
in childhood (Mckenna Brodsky, 2005)
Repeated bouts of asthma, bronchitis, non-
specific reactive airway disease, croup, and
pneumonia may all indicate airway prob-
lems, GI tract dysfunction, or swallowing
impairments. Knowledge of previous sur-
geries may establish any prior manipulations
of the airway or aerodigestive tract such as a
tracheoesophageal fistula (TEF) repair.
The environmental history provides
information about potentiating irritants,
such as secondhand tobacco smoke or
contributory dietary habits. Social history
will help to establish the caregiver support
available to the patient. Home-based airway
support often requires enormous resources
including around-the-clock nursing, sup-
plies, and special equipment. Single-parent
families, group homes, and the presence of
relatives with other health problems may
influence treatment recommendations.
The review of systems should be thor-
ough but particularly focused on the child’s
airway, GI tract, and neurodevelopment
that includes speech and language acquisi-
tion. For neonates and infants, questions
must be asked regarding breathing patterns,
with the infant in quiet repose or asleep in
different positions, and in varied positions
while crying and feeding. The parent’s or
primary caregiver’s description of respi-
ratory difficulty and airway noises often
provides invaluable information. Inquiry
is made regarding possible associated signs
and symptoms such as cough, stridor, ster-
tor and “noisy breathing,” wheezing, gur-
gling or “wet” sounds with respiration, cya-
nosis, breath-holding or apnea (cessation of
breathing for 8 s or more), snoring, hoarse-

4. The Upper Airway and Swallowing 151
ness or voice changes, opisthotonus (back
arching), or feeding refusal.
Establishment of a diagnosis is helped
by using symptoms and signs to local-
ize the etiology to a specific anatomic
site (Table 4–1). Stridor, the term used to
describe a high-pitched turbulent airflow
through the larynx or trachea, may be pres-
ent during inspiration, expiration, or both.
Stridor is not a diagnosis itself but rather
an indication of an airway narrowing or
obstruction. It is never a normal finding and
always warrants further evaluation. Some-
times stridor is mistakenly characterized as
wheezing, often resulting in inappropriate
and unsuccessful treatment directed at the
trachea, bronchi, or lungs and, therefore,
ineffective in the upper airways. Other
upper airway sounds to note are nasal con-
gestion, particularly when snorting (or
stertor1
) is present. Stertor is a low-pitched
snorting or grunting sound that usually
indicates a partial obstruction of the nose
or pharynx. Gurgling with respiration is
associated with pooling of secretions in the
hypopharynx, pyriform sinuses, or laryn-
geal inlet, often seen in children with focal
or global neurodevelopmental delay or
EERD. Cough can be indicative of laryngeal
penetration or aspiration occurring prior to,
during, or immediately after a swallow (see
Chapter 8). The absence of a cough does not
eliminate penetration or aspiration from the
differential diagnosis of a swallowing and
feeding problem. Silent aspiration, that is
aspiration without a cough or other observ-
able response, occurs in children with and
without neurologic impairment (Arvedson,
Rogers , Buck, Smart, Msall, 1994; Lefton-
Greif, Carroll, Loughlin, 2006). A weak or
hoarse cry should alert the practitioner to
potential laryngeal involvement, including
inflammatory disease such as EERD, or ana-
tomic–physiologic problems such as vocal
fold nodules, injury from intubation, and
impaired vocal fold mobility. Apnea may
be associated with central nervous system
(CNS) abnormalities, GERD, or anatomic
obstruction.
The physical examination of the pediatric
airway begins with an overall assessment
of the infant or child’s appearance for signs
suggesting respiratory distress, poor nutri-
tion or neurologic abnormalities such as
hypotonia, abnormal posture, and poor head
control. Craniofacial or other congenital
anomalies are noted and, if present, should
prompt consultation with a geneticist or
dysmorphologist (see Chapter 12).
Airway assessment begins with observa-
tion of the patterns of respiration, including
resting respiratory rate and the presence
of abnormal upper airway noises, before
any oral feeding observation is made or
attempted. Examination should also include
auscultation over the larynx and tracheal
for airway sounds of stridor that may not
be overtly audible. Airway sounds sug-
gest narrowing or obstruction of the air-
way which may occur at any level of the
upper aerodigestive tract from the nose
to the tracheobronchial tree, or esopha-
gus. Esophageal abnormalities can extrin-
sically compress the airway and present
with stridor. Stertor and stridor, the most
common respiratory signs associated with
1
The terms snorting and stertor have been used interchangeably. Some clinicians include both under the
general heading of stridor. However, stridor and stertor are different sounds emanating from different
anatomic locations in the upper airway. Implications for diagnosis and treatment are likely to be different.

152
Table 4–1. Abnormalities of Upper Aerodigestive Tract With Airway Presentation
Location Abnormality Clinical Presentation
Nose/nasopharynx Choanal stenosis/atresia Nasal obstruction/discharge
Stridor and cyanosis with feeding,
relieved by crying
Tumors Nasal obstruction
Stertor with feeding
Deviated septum Traumatic birth
Nasal obstruction
Stertor with feeding
Midface hypoplasias Craniofacial anomalies—Crouzon
and Apert
Stertor increased with feeding
Oral cavity/oropharynx Cleft lip, palate, or both Air gulping, snorting, choking
Nasopharyngeal regurgitation
Ineffective suck
Mandibular hypoplasias Craniofacial anomalies—
Robin sequence, hemifacial
microsomias, Treacher Collins
(Chapter 12)
Choking and grunting that
increase with feeding
Adenotonsillar hyperplasia Obstructive sleep apnea with
dysphagia
Undernutrition/malnutrition
Hypopharynx Muscular hypotonia Stertor
Nasopharyngeal/ hypopharyngeal
collapse on FFNL
Laryngeal Laryngeal/subglottic
stenosis
Stridor at rest that increases with
feeding
Hoarseness and sometimes
cough
Laryngeal clefts Coughing with feeding
Stridor may be present at rest or
increased with feeds
Vocal fold paralysis Hoarseness
Stridor
Choking on feeds
Laryngomalacia Stridor at rest and with feeds
Symptoms of EERD (Chapter 5)

Location Abnormality Clinical Presentation
Laryngeal continued Tracheobronchomalacia Expiratory stridor/wheeze
Apnea
Cyanosis
Increased work of breathing
during feeds
Supraglottic edema
(secondary to EERD)
Stridor
Hoarseness
Food refusal
Difficult and slow feeds
Esophagus Tracheoesophageal fistula Cough, cyanosis, and choking
with feeding
Recurrent pneumonia
Esophageal mass Dysphagia
Stridor
Mediastinum Vascular anomalies
(aberrant right subclavian,
double aortic arch, right
aortic arch with left
ligamentum)
Feeding difficulties
Expiratory stridor/wheeze
Mediastinal tumors/cysts Dysphagia
Expiratory stridor/wheeze
Note. EERD = extra-esophageal reflux disease; FFNL = flexible fiberoptic nasopharyngolaryngoscopy.
swallowing and feeding problems, may be
accompanied by suprasternal, substernal,
and intercostal retractions, which signal a
significantly increased work of breathing. In
such instances, caloric intake may become
inadequate for optimal growth and develop-
ment due to the added caloric expenditure
associated with the work of breathing. An
assortment of primary cardiac and pul-
monary diseases may also cause increased
work of breathing, feeding problems and
undernutrition.
Assessment of an infant’s cry or child’s
voice may reveal hoarseness or dysphonia.
Such findings may indicate an abnormal-
ity in vocal fold approximation. A history
of endotracheal intubation or aerodiges-
tive tract manipulation increases potential
for abnormalities of the vocal fold edge or
mobility and airway stenosis. The presence
of cough may indicate penetration, aspira-
tion, tracheo-esophageal communication in
addition to primary cardiopulmonary, neu-
rologic or behavioral etiologies.
It cannot be overemphasized that the
clinician evaluating the airway must make
firsthand observation of the patient at rest
and in multiple positions while crying, as

well as during feeding. The presence of overt
airway obstruction should prompt imme-
diate examination of the airway. Signs of
airway distress are often absent until feeds
are introduced and may include snorting,
grunting, head bobbing, an increased rate of
breathing (tachypnea) and frequent pauses
between swallows that may indicate a con-
comitant airway problem.
When oral feeding is associated with
significant airway distress, such as severe
coughing, cyanosis, apnea, bradycardia or
choking, it may need to be discontinued
until the etiology is determined, the prob-
lem resolves (e.g., infection, obstruction), or
the airway distress is stabilized, and swal-
lowing may be assessed. The triad of chok-
ing, coughing, and cyanosis that occurs
with oral feeding is most commonly seen
in unrecognized tracheoesophageal fistula
(TEF) or laryngeal cleft, particularly in an
infant who has had recurrent pneumonia
during the first few months of life. When a
high-pitched stridor in infants coexists with
coughing, choking, and cyanosis, laryngo-
malacia should be considered as well.
Craniofacial findings may include man-
dibular hypoplasia (Figure 4–1) or asym-
metry seen in hemifacial microsomia,
indicating abnormal soft tissue structures
or tongue position that may interfere with
feeding. Feeding difficulties can occur
with various degrees of palatal clefting see
(Chapter 12). Identification of a submucous
cleft palate (Figure 4–2) requires intraoral
examination for bifid uvula, a zona pellu-
cida (submucosal absence of the muscula-
ris uvulae), and notching of the hard palate.
Figure 4–1. A. Infant with micrognathic mandible from an isolated Pierre Robin
sequence. B. U-shaped cleft palate also characteristic of Pierre Robin sequence.
(Source: From Volk, M. S., Arnold, S., Brodsky, L. [1992]. Otolaryngology and
audiology. In L. Brodsky, L. Holt, D. H. Ritter-Schmidt (Eds.), Craniofacial
anomalies: An interdisciplinary approach [p. 169]. St. Louis, MO: Mosby-Year
Book. Copyright 1992 by Mosby-Year Book. Reprinted by permission.)
A B

However, all of these physical findings may
be absent with nasopharyngeal regurgita-
tion during feeding occurring as the only
sign of an occult submucous cleft palate or
velopharyngeal incompetence, and identi-
fication requires endoscopic visualization
of the nasal side of the palate. Findings of
a groove in the soft palate rather than the
usual bulge in the soft palate normally
created by contraction of the muscularis
uvulae during palatal elevation. Restricted
lingual frenulum with or without an upper
lip tie may also interfere with feeding, par-
ticularly breastfeeding. Attention must be
given to the potential of airway obstruction
(Genther, Skinner, Bailey, Capone, Byrne,
2015). Also see Chapters 7 and 9.
Instrumental Evaluation
of the Upper Airway
Flexible fiber-optic nasopharyngolaryn-
goscopy (FFNL2
) is essential to complete
an examination of the upper aerodigestive
tract in infants and children with swallow-
ing and feeding disorders (Figure 4–3).
Visualization of structures from the anterior
nares, posteriorly through the nasal cavity
into the nasopharynx, and inferiorly into
the hypopharynx and the larynx, provides
both structural and functional information
regarding the upper airway. FFNL is par-
ticularly useful in determining functional
aspects of airway dynamics, such as palatal
closure, vocal fold function, tongue place-
ment during inspiration and expiration,
and the presence and degree of collapse
of the nasopharynx or hypopharynx (see
Table 4–1). Fiberoptic endoscopic evalu-
ation of swallowing (FEES) can be useful
for a dynamic evaluation to observe for
laryngeal penetration, aspiration, pharyn-
geal swallow function, and cough of chil-
dren with dysphagia. (See Chapter 8.) This
procedure is also helpful for children with
upper airway abnormalities such as laryn-
gomalacia, laryngeal cleft, and vocal fold
immobility or dysmotility.
Radiologic evaluation of the airway may
include a posterior-anterior (PA) and lateral
radiograph of the airway, performed with
high KV filter to highlight soft-tissue/airway
interfaces. Findings may indicate subglottic
Figure 4–2. Soft palatal defect with intact
overlying mucosa comprising two of the fol-
lowing three findings: (1) notching of the
posterior border of the hard palate, (2) bifid
uvula, or (3) muscular diastasis leading to a
midline translucent zone or furrow in the soft
palate. Submucous cleft palate. Note the cleft
uvula and the translucent indentation of the
soft palate. (Source: National Human Genome
Research Institute. Palate, submucous cleft.
https://elementsofmorphology.nih.gov/index.
cgi?tid=30b9e9da9758d9d7)
2
The terms flexible fiberoptic nasopharyngoscopy, flexible fiberoptic nasopharyngolaryngoscopy, and flexible
fiberoptic laryngoscopy are used interchangeably.

or hypopharyngeal abnormalities. Chest
x-ray, barium esophagram, and ultrafast
dynamic and static computed tomographic
(CT) scans of the chest may provide fur-
ther information valuable in diagnosis and
treatment (Brody, Kuhn, Seidel, Brodsky,
1991; Stagnaro, Rizzo, Torre, Cittadini,
Magnano, 2017).
When an abnormality of the endolar-
ynx, subglottis, trachea, bronchi, or esoph-
agus is suspected, evaluation using a rigid
laryngoscope, bronchoscope, or esopha-
goscope under a general anesthesia is per-
formed. Rigid endoscopy under anesthesia
is necessary to evaluate for subglottic ste-
nosis, laryngotracheal cleft, tracheoesopha-
geal fistula, chronic tracheobronchitis from
EERD, and tracheobronchomalacia. Flex-
ible bronchoscopy can be used to evaluate
distal airway disease and lavage bronchi
for cultures.
Masses in the mediastinum, neck, and
esophagus, as well as vascular anomalies of
the great vessels, can also present with simul-
Figure 4–3. Infant undergoing flexible fiberoptic laryngoscopy to
evaluate the upper airway (e.g., laryngomalacia). Digital record-
ing (in upper right corner) allows storage, review in real time, and
slow motion, as well as retrieval of multiple examinations over
time for comparison purposes.

taneous feeding and breathing problems.
CT scan with contrast, magnetic resonance
imaging (MRI), echocardiography, or angi-
ography may be indicated in select cases.
Specific Airway and
Feeding Problems
Gastroesophageal Reflux
Disease (GERD) and
Extra-Esophageal Reflux
Disease (EERD)
Infants and older children who have air-
way distress when feeding or eating may
also suffer from GERD/EERD.3
Common
manifestations of GERD/EERD can be
noted in the GI tract, airway, and other sys-
tems (Table 4–2 and see Chapter 5). Regur-
gitation of feeds is common in the young
infant and is not necessarily regarded as
pathologic, that is, requiring evaluation
and treatment, unless it causes other signs
and symptoms that have a negative impact
on the health and well-being of the child.
GERD/EERD that alters normal feeding
and eating patterns or contributes to airway
distress or other otolaryngologic problems
is by definition considered pathologic reflux
and requires evaluation and treatment.
Airway symptoms are present in approxi-
mately 43% of children with GERD/EERD
(Andze, Brandt, St. Vil, Bensoussan,
Blanchard, 1991). Tracheal mucosa may
reveal cobblestoning, blunt carina, and loss
of tracheal ring architecture, all potential
signs of chronic aspiration (Figure 4–4)
(Carr et al., 2000). Conversely, the percent-
age of children with GERD/EERD-induced
respiratory problems is about 30% (Bau-
man, Sandler, Smith, 1996). The children
at greatest risk include those who have neu-
rologic impairment, anatomic abnormali-
ties (e.g., TEF and hiatal hernia), motility
disorders, and hyperactive airways.
The anatomic and physiologic protec-
tive mechanisms that play a role in prevent-
ing GERD/EERD are described in Chapters
2 and 5. Complications from GERD, such
as erosive esophagitis, stricture formation,
undernutrition, and malnutrition are well
known and occur in about 15% of untreated
cases (Bauman et al., 1996). Refluxate that
contains acid, enzymes, bacteria, and par-
tially digested food has an unpredictable
effect on the upper esophagus, larynx, and
lower airways.
Respiratory diseases may exacerbate or
cause GERD/EERD (Gaude, 2009; Oren-
stein Orenstein, 1988; Pearson et al., 2011).
Reactive airway disease, asthma, broncho-
pulmonary dysplasia, recurrent bronchitis,
cystic fibrosis, and central alveolar hypoven-
tilation syndrome are all associated with
GERD/EERD (Andze et al., 1991; Bauman
et al., 1996; Gaude, 2009; Halstead, 1999;
Orenstein Orenstein, 1988). Alterations
in thoracoabdominal pressure relationships
occur secondary to both forced expiration
(cough, wheeze) and forced inspiration
(stridor, stertor, hiccups). The associated
increase in intra-abdominal pressure delays
gastric emptying and increases the poten-
tial for gastric contents to back-flow into
the esophagus. Both mechanisms result in
refluxate being drawn from the stomach
into the esophagus and upper airway.
Intractable cough, uncontrollable reac-
tive airway disease, recurrent airway dis-
tress, and recurrent airway infections may
have contributions from GERD/EERD,
3
GERD/EERD is not necessarily a diagnosis in and of itself but may indicate an underlying disease process.
Determination of a specific diagnosis is important if successful treatment is to occur.

158
Table 4–2. Common Manifestations of Gastroesophageal Reflux Disease
(GERD) and Extra-Esophageal Reflux Disease (EERD)
Common Manifestations Signs And Symptoms
Frequent gastrointestinal
manifestations
Frequent emesis
Burping
Abdominal pain
Heartburn
Difficulty swallowing
Undernutrition/malnutrition
Chest pain
Regurgitation of food
Rumination
Frequent swallowing
Picky or slow eater
Food refusal
Frequent airway manifestations Hoarseness
Cough
Throat clearing
Recurrent croup
Recurrent pneumonia
Asthma exacerbations
Stridor/stertor
Apnea
Obstructive or central sleep apnea
Hiccups
Gurgly respirations
Brief resolved unexplained event (BRUE)
Occasional manifestations Halitosis
Torticollis (Sandifer’s syndrome)
Opisthotonos (back arching)
Drooling
Chronic sore throat
Globus sensation
Morning nasal congestion/cough
Frequent nocturnal awakenings
Otalgia
Tooth enamel erosion
Intractable rhinosinusitis
Chronic otitis media
Hypogeusia (loss of taste)

and the clinician must balance the risk and
benefit of GERD treatment in children (see
Chapter 5).
Choanal Atresia and
Nasal Stenosis
Choanal Atresia
Congenital choanal atresia, which blocks the
posterior nasal airway flow, affects approxi-
mately 1:5,000 to 1:8,000 births, twice as
prevalent in females as it is in males (Cedin,
Atallah, Andriolo, Cruz, Pignatari, 2012)
and usually presents with the first feeding.
Choanal atresia may be bilateral or unilat-
eral, with unilateral choanal atresia occur-
ring in about 50% to 60% of cases. Almost
all infants are, if not obligate, at least pri-
marily nasal breathers for about the first
6 months of life. Classically, infants with
bilateral choanal atresia have cyclical peri-
ods of cyanosis at rest, which are relieved
when the child becomes agitated, begins to
cry, and breathes through the mouth, thus
bypassing the airway obstruction. A return
to the resting, noncrying state ensues, and
the cycle begins anew. Surprisingly, some
of these infants may have little or no respi-
ratory distress at rest but become severely
compromised (with cyanosis) at the onset of
oral feeding. Nasal patency is first evaluated
by inspection of the anterior nares. Check-
ing for airflow using a wisp of cotton created
at the end of a cotton-tipped applicator or
fogging a mirror held at the nares readily
provides information regarding airflow.
Passing catheters through the nose into
the nasopharynx can help to evaluate nasal
patency. However, subtle forms of nasal or
choanal stenosis may be missed with this
technique. The gold standard is flexible
nasopharyngoscopy to evaluate for choanal
atresia. Some children have choanal atresia
as part of an association of multiple congen-
ital anomalies, for example, CHARGE syn-
drome (Coloboma, Heart, Atresia choanae,
Figure 4–4. Severe tracheal cobblestoning associated with reflux-
induced chronic aspiration and presenting as chronic cough.

Retardation, Genitourinary, and Ear abnor-
malities). Children with CHARGE syn-
drome almost always have neurodevelop-
mental delays associated with decreased
neuromuscular tone and incoordination, all
further complicating feeding. Tracheostomy
offers the most effective means for managing
the airway in some of these children (Asher,
McGill, Kaplan, Friedman, Healy, 1990).
Others may require long-term gastrostomy
tube feeding supplements (Dobbelsteyn,
Peacocke, Blake, Crist, Rashid, 2008).
Inspection of both posterior nasal cho-
anae using FFNL with a 2-mm or 3-mm
endoscope or a 2.7-mm rigid telescope is
most helpful. Direct visualization, combined
with axial computed tomographic scan of
the nose and nasopharynx, will determine
the type (membranous versus bony) and
the extent of the lesion (Figure 4–5). Sur-
gical repair of bilateral choanal atresia is
usually performed in the neonatal period
(Figure 4–6). The transnasal approach is
preferred in this age group by most (Eladl
Khafagy, 2016; Gulşen et al., 2017; Lantz
Birck, 1981; Richardson Osguthorpe,
1988). Transnasal approach with short-term
stenting was not found to decrease the inci-
dence of reclosure and restenosis of the pos-
terior choanae, but did have higher compli-
Figure 4–5. Axial computed tomographic scan
showing bilateral bony choanal atresia. (Source:
From Volk, M. S., Arnold, S., Brodsky, L. Otolar-
yngology and audiology. In L. Brodsky, L. Holt, D.
H. Ritter-Schmidt [Eds.], Craniofacial anomalies: An
interdisciplinary approach [p. 172]. St. Louis, MO:
Mosby-Year Book. Copyright 1992 by Mosby-Year
Book. Reprinted by permission.)

cation rates that included granulation tissue
and dislodgement (Saafan, 2013). Topical
mitomycin is reported to be efficacious as
an adjuvant therapy with less granulation
tissue, lower rate of restenosis, and fewer
surgeries, whereas stenting is associated
with more procedures, greater formation
of granulation tissue, and longer overall
hospital stays (Carter, Lawlor, Guarisco,
2014). However, Carter and colleagues rec-
ommend consideration to stent placement
in all neonates for the prevention of postop-
erative obstruction. Transpalatal repair with
long-term stenting has been indicated in the
Figure 4–6. Pre- and post-repair of choanal atresia. A. Nasal
telescopic view of a blind pouch at the posterior nasal choa-
nae, consistent with choanal atresia. B. Repaired right cho-
anal atresia.
B
A

past for revision surgery or in other cases
where the anatomy is not favorable for the
transnasal approach (Maniglia Goodwin,
1981). Cedin and colleagues (2012) found
no definitive evidence (no randomized
controlled trials identified in a Cochrane
review) to demonstrate potential advan-
tages and disadvantages of any specific sur-
gical technique for choanal atresia. They
urge multicenter randomized controlled
trials to test both effectiveness and safety of
different surgical techniques.
Nasal Stenosis
Stenosisorobstructionfromadeviatednasal
septum secondary to birth trauma should
be either defined or ruled out (Emami,
Brodsky, Pizzuto, 1996). Anterior and
posterior rhinoscopy may be accomplished
using an otoscope with nasal speculum
attachment. Edema of the nasal mucosa is
common in children who undergo nasal
suction at birth. The edema resolves with
cessation of suctioning. Conservative man-
agement for some infants with congenital
nasal obstruction may be adequate. These
interventions include, but are not neces-
sarily limited to, suctioning and humidifi-
cation along with medical therapies (e.g.,
intranasal drops and nasal sprays) (Patel
Carr, 2017). Topical decongestants such
as Neo-Synephrine 1/8% may be used for
a few days, and sometimes prolonged use
of steroid nose drops will be useful (Derkay
Grundfast, 1991). Midnasal stenosis and
pyriform aperture stenosis may also cause
obstructive symptoms and associated feed-
ing problems (Knegt-Junk, Bos, Berkov-
its, 1988; Sultan, Lefton-Greif, Brown,
Ishman, 2009).
Pyriform aperture stenosis is also
amenable to surgical correction followed
by stenting for 7 to 10 days (Devambez,
Delattre, Fayoux, 2009). Bazak, Ibrahim,
Hussein, Abdelnaby, and Elwany (2018)
described a treatment modality using
extramucosal pyriplasty with decompres-
sion of the nasolacrimal duct (NLD) with-
out stenting. This procedure provides relief
of nasal obstruction while avoiding draw-
backs of stenting and shortcomings of the
conservative methods.
These children should have a full neu-
rologic workup preoperatively due to other
associated abnormalities such as pituitary
and brain defects. Severe midnasal steno-
sis often requires multiple revision dila-
tions and possibly even tracheostomy to
bypass the obstruction until the child grows
enough to support the airway orally.
Craniofacial Anomalies
Midface Hypoplasias
The most common midface hypoplasias
are associated with the craniosynostoses
of Crouzon and Apert. Phenotypic pre-
sentation is highly variable for these auto-
somal, dominantly inherited craniofacial
anomalies in about 8% with the remainder
occurring as a spontaneous isolated defect
(Governale, 2015). Typically they are char-
acterized by cranial synostosis (premature
closure of the cranial sutures), midface
hypoplasia, ocular proptosis due to shal-
low orbits, cleft palate, and, in the case
of the Apert syndrome, finger and hand
abnormalities (Figures 4–7 and 4–8). In the
more severe forms, especially when a cleft
palate is not present, stridor at rest is com-
mon. Lateral neck radiographs may reveal
a maxilla that is literally impacted against
the skull base (Figure 4–9). In less severe
forms of maxillary retrusion, oral feeding
may stress a marginally compensated air-

way. Surgical treatment may occur soon
after diagnosis (or later for some). Surgi-
cal methods include open calvarial recon-
struction, minimally invasive strip craniec-
tomy with postoperative molding helmet,
minimally invasive strip craniectomy with
spring implantation, and cranial distrac-
tion (Governale, 2015). Müller-Hagedorn
and colleagues (2018) reported treatment
of airway obstruction with a modified Tub-
ingen Palatal Plate (TPP) as mostly effective
and safe. They emphasized the need for pro-
spective studies that may help avoid more
invasive procedures, such as tracheostomy,
for some children until the diameter of the
airway increases with growth. The airway
may be improved with midface advance-
ment, a procedure that has been performed
in some cases as early as age 3 years, but is
best deferred until after puberty.
Mandibular Hypoplasias
The Pierre Robin sequence classically has
been described by a triad of clinical signs
to include mandibular hypoplasia, micro-
gnathia, glossoptosis (backward, downward
placement of the tongue) (Figure 4–10), and
a U-shaped cleft palate (see Figure 4–1).
This condition is now labeled Pierre Robin
sequence or syndrome with signs described:
micrognathia, glossoptosis, and obstruction
of the upper airways frequently associated
with a palatal cleft (e.g., Cladis et al., 2014;
Figure 4–7. A. Frontal view of an infant with Apert syndrome. Note midface hypo-
plasia in the infant and syndactyly of the affected parent holding the child. B. Lateral
view of infant with Apert syndrome. (Source: From Volk, M. S., Arnold, S., Brod-
sky, L. Otolaryngology and audiology. In L. Brodsky, L. Holt, D. H. Ritter-Schmidt
[Eds.], Craniofacial anomalies: An interdisciplinary approach [p. 172]. St. Louis, MO:
Mosby-Year Book. Copyright 1992 by Mosby-Year Book. Reprinted by permission.)
A B

164
Figure 4–8. Midface hypoplasia in children with Apert syndrome. Note underde-
velopment of the infra-orbital and peri-alar regions leading to more pronounced
concavity of the face and reduced nasolabial angle. This gives the appearance of
prognathia. (Source: Elements of Morphology, National Human Genome Research
Institute.)

165
Figure 4–9. Lateral neck radiograph of infant in Figure 4–7.
Note maxilla impacted on skull base and the absence of a
nasopharynx.(Source: From Volk, M.S., Arnold, S., Brodsky,
L. Otolaryngology and audiology. In L. Brodsky, L. Holt, D. H.
Ritter-Schmidt [Eds.], Craniofacial anomalies: An interdisciplin-
ary approach [p. 172]. St. Louis, MO: Mosby-Year Book. Copy-
right 1992 by Mosby-Year Book. Reprinted by permission.)
Figure 4–10. Glossoptosis. Note the tongue’s posterior placement
in the oral cavity and the presence of the formula. (Source: https://
elementsofmorphology.nih.gov/index.cgi?tid=ddc1a2c7e23644e8)

Giudice et al., 2018). Some children have
airway obstruction at rest that includes stri-
dor, retractions, and cyanosis. In other chil-
dren, obstruction may be subtle in its pre-
sentation and not manifest until feeds are
introduced. Grunting, choking, and cough-
ing with prolonged, difficult feeds may indi-
cate airway compromise. The mechanisms
can vary among patients, but three basic
mechanisms have been described and can
be identified using FFNL. The most com-
mon mechanism for obstruction is glos-
soptosis at the level of the hypopharynx
during inspiration. On occasion, the palatal
shelves of the cleft may be drawn medially
to obstruct the airway. At other times, lat-
eral pharyngeal wall hypotonia may cause
pharyngeal/hypopharyngeal collapse (Giu-
dice et al., 2018; Shprintzen, 1988).
Treatment of the airway obstruction
in nonsyndromic Pierre Robin sequence
depends on the anatomic location of the ob-
struction. Treatment options (Khansa et al.,
2017) include watchful waiting for growth
and development in mildly affected cases,
nasopharyngeal tubes, stenting, prone posi-
tioning (Delorme, Laroque, Caouette-
Laberge, 1989), glossopexy (tongue-lip
adhesion [TLA]) (Argamaso, 1992; Great-
house et al., 2016; Viezel-Mathieu, Safran,
Gilardino, 2016), mandibular distrac-
tion osteogenesis (MDO; Figure 4–11)
(Breik, Umapathysivam, Tivey, Ander-
son, 2016; Jenny, Massenburg, Weissler,
Taub, 2017; Khansa et al., 2017), and tra-
cheostomy. Multiple reports of outcomes
following TLA, MDO, and conservative
management stress that patient selection
to determine surgical need and the most
appropriate surgical procedure is a critical
factor in comparing outcomes. Overall, it
appears that MDO demonstrates superior
outcome measures at 1 month and 1 year
compared to TLA (Flores et al., 2014; Great-
house et al., 2016). Papoff and colleagues
(2013) found that infants with severe air-
way obstruction related to PRS can benefit
safely from either TLA or MDO. MDO sta-
bilizes airway patency more efficiently with
full oral feeding achieved more rapidly than
with TLA. It is important to note that not
all mandibular hypoplasias are manifesta-
tions of the Pierre Robin sequence. Accurate
diagnosis is essential for the development of
long-term treatment plans and for predict-
ing prognosis. A genetics or dysmorphology
Figure 4–11. Pre- and post-mandibular distraction osteogenesis (MDO) for mandibular
hypoplasia manifestations of Pierre Robin sequence.The infant was fed by nasogastric
tube before distraction. (Source: Courtesy of Jordan Steinberg, MD.)
A B

evaluation is necessary in every case. An
important example is PRS as part of Stick-
ler syndrome, which is characterized with
a high probability of retinal detachment
(Mingo et al., 2016; Vilaplana, Muiños,
Nadal, Elizalde, Mojal, 2015). Treatment
depends on etiology and may vary consider-
ably among patients.
Infants with other craniofacial anoma-
lies that include genetic syndromes and
neurologic disease often present with, or
encounter, feeding difficulties. These condi-
tions are discussed in depth in Chapter 12.
Tracheoesophageal Fistula
Tracheoesophageal fistula (TEF) may be
either congenital or acquired. The acquired
forms follow trauma, foreign-body inges-
tion, or are a complication of surgery, such
as tracheotomy. As mentioned previously,
the triad of coughing, choking, and cya-
nosis is common. Recurrent pneumonia,
particularly in the first 6 months of life, is
another presenting sign. Several types of
tracheoesophageal fistulae are described
(Figure 4–12). Those presenting with esoph-
ageal atresia usually present with polyhy-
dramnios in the mother and total inability
of the infant to swallow that is noted dur-
ing the first attempt to feed orally. In an
H-type fistula, barium esophagram may
reveal a tract from the esophagus into the
trachea (Figure 4–13); however, rigid endo-
scopic evaluation of the esophagus and tra-
cheobronchial tree usually is required for
a comprehensive evaluation of this type
of TEF. There should be clinical suspicion
for an H-type fistula if there is a history
of desaturations with oral feedings and
recurrent aspiration pneumonia. Surgical
repair is required, but esophageal stenosis
at the operative site may cause continued
dysphagia and necessitate adjustment of
diet and repeated esophageal dilatations.
Figure 4–12. A. Distal tracheoesophageal fistulae are most commonly associated with proxi-
mal esophageal atresia. B. Esophageal atresia without tracheal connection. C. Of this group of
anomalies, tracheoesophageal fistula alone, known as an H-type fistula, is the least common.

Tracheomalacia and/or bronchomalacia
may also be present after repair of the TEF
and may result in or contribute to continued
feeding difficulty, stridor, and respiratory
distress. The combination of a collapsed
trachea from the tracheomalacia and the
presence of a food bolus in the esophagus
can further extrinsically push on the poste-
rior tracheal wall. Recurrent TEF must be
ruled out in children with recurrent or per-
sistent feeding and respiratory symptoms
with a history of TEF (Figure 4–14). Laryn-
geal cleft is commonly associated with TEF
and may further contribute to dysphagia
with possible aspiration in these children.
Hseu reviewed 430 patients with TEF/EA
finding 21% had vocal fold immobility, 25%
had laryngeal cleft, and 37% tracheomalacia
(Hseu, Recko, Jennings, Nuss, 2015). The
presence of vocal fold immobility, tracheo-/
bronchomalacia, and laryngeal cleft should
be considered in TEF patients who continue
to be symptomatic following repair.
Figure 4–13. Barium esophagram reveals an
H-type tracheoesophageal fistula with con-
trast in both the esophagus (large arrow) and
trachea (double arrow) with a well-defined
connection (small single arrow).
Figure 4–14. Residual tracheal esophageal fistula.

Tracheomalacia Apart from TEF
Tracheomalacia may also be secondary to
external compression from vascular rings
and slings. Complete vascular rings are
secondary to double aortic arches from
the heart or a right aortic arch with retro-
esophageal left subclavian artery and left
ligamentum arteriosum. Both complete
vascular rings encompass the esophagus
and trachea leading to tracheal and esopha-
geal compression. Patients may show both
airway and dysphagia symptoms. Incom-
plete vascular rings do not encompass both
the trachea and esophagus and do not lead
to the significant airway symptoms and
dysphagia that complete rings do. A vas-
cular sling is an aberrant pulmonary artery
causing posterior tracheal compression
and anterior esophageal compression. Sur-
gical treatment via a thoracic approach is
the standard procedure for these vascular
rings and slings with possible relief of the
extrinsic compression of the trachea at
the same time via tracheal suspension or
aortopexy.
Laryngeal Anomalies
A wide variety of laryngeal abnormalities
may present with stridor and feeding prob-
lems, including laryngomalacia, vallecular
cyst, laryngeal webs, epiglottic structural
abnormalities (absent epiglottis) (Bonilla,
Pizzuto, Brodsky, Brody, 1998; Reyes,
Arnold, Brooks, 1994; Rutter, 2014), and
posterior laryngeal clefts all can lead to
feeding difficulties associated with airway
obstruction as the initial clinical presenta-
tion. Choking and coughing soon after a
feeding is initiated may occur, and these
conditions can mimic TEF. Infants with
vocal fold paralysis, chronic reflux laryn-
gitis (with or without laryngomalacia), and
subglottic stenosis may have significant
difficulty with both breathing and feeding.
FFNL and rigid direct laryngoscopy pro-
vide different sets of information needed to
arrive at an accurate diagnosis.
Vocal Fold Paralysis
Bilateral vocal fold paralysis may be due to
idiopathic, neurologic (e.g., Arnold-Chiari
malformation), or even secondary to sur-
gery or prolonged intubation. Diagnosis is
made by awake laryngoscopy and suspen-
sion laryngoscopy and bronchoscopy to rule
out other etiologies, such as posterior glot-
tic stenosis. Nearly two-thirds of patients
may need a tracheostomy due to stridor
and airway obstruction of the paramedian
vocal folds (Funk, Jabbour, Robey, 2015).
Of those patients with bilateral vocal fold
paralysis and tracheostomy about two-
thirds will be decannulated as well. Dura-
tion of tracheostomy may vary considerably
from one child to the next.
Unilateral vocal fold paralysis may be
secondary to birth trauma, cardiac abnor-
malities, prior surgery, prolonged intuba-
tion, or idiopathic. However, one of the
most common etiologies is following car-
diac surgery, with or without a history of
extracorporeal membrane oxygenation
(ECMO) (Schumacher, Weinfeld, Bartlett,
1989). Many of these children present with
dysphonia, respiratory symptoms, and dys-
phagia. Spontaneous recovery varies from
3% to 45% with a significant proportion
having aspiration as well. A large propor-
tion of these patients may need tracheos-
tomy tube (25%) for breathing and gas-
trostomy tube (40%) to meet nutritional
needs (Jabbour, Martin, Beste, Robey,
2014; Nichols et al., 2014; Truong et al.,
2007). Many infants with vocal fold motion
impairment (VFMI) after complex aortic
arch reconstruction show improvement in

VFMI within 5 months of surgery and com-
plete resolution an average of 10.5 months
after surgery (Rodney, Thompson, Ander-
son Burkhart, 2019).
For children with aspiration and good
prognosis for swallowing and sensation, a
vocal fold medialization procedure may be
considered to improve glottic closure and
cough, and decrease aspiration. FEES may
be helpful in the assessment of the glottic
closure, and injection laryngoplasty can
be considered even in the neonatal period.
However, the improved medialization must
be balanced with the possibility of increased
airway obstruction in young infants.
Vallecular Cyst
Vallecular cyst may present with stridor,
dysphagia, or with an acute life-threatening
event (Figure 4–15). Diagnosis is by nasal
laryngoscopy and surgical excision, or mar-
supialization is the treatment (Tsai, Lee,
Fang, Li, 2013). These children are not
expected to have ongoing dysphagia follow-
ing surgical excision.
Laryngeal Web
Laryngeal web is a rare congenital disease
associated with stridor and feeding and
breathing difficulties (Figure 4–16). Laryn-
geal anomalies, including laryngeal web,
are common in children with 22q11.2 dele-
tion syndrome (Leopold, De Barros, Cel-
lier, Drouin-Garraud, Dehesdin, Marie,
2012) , which has association with not only
cardiac abnormalities, but cleft palate as
well. Of note, 22q11.2 deletion syndrome
was previously called DiGeorge syndrome,
velocardiofacial syndrome, or CATCH22
syndrome. See Chapter 12.
Laryngomalacia in Infants
Infants presenting with inspiratory stridor
will have a diagnosis of laryngomalacia in
75% of cases. Laryngomalacia is charac-
terized with inspiratory stridor secondary
to foreshortened aryepiglottic folds and
redundant arytenoid mucosa that flops into
the airway with inspiration (Figure 4–17).
Etiology is unclear but may be secondary
Figure 4–15. Vallecular cyst.

171
Figure 4–17. A child with severe laryngomalacia with the
omega-shaped epiglottis and prolapsing and obstructing pos-
terior arytenoid mucosa.
Figure 4–16. Laryngeal web fusing 50% of the anterior true
vocal folds.

to poor neurogenic tone of the supraglottic
tissue (Thompson, 2007, 2010). Laryngo-
malacia is characterized as mild, moder-
ate, and severe dependent on the severity
of obstruction of the glottis. A diagnosis of
laryngomalacia can only be confirmed with
awake flexible laryngoscopy.
Findings on presentation that should
prompt more urgent otolaryngologic eval-
uation are apnea, tachypnea, cyanosis, fail-
ure to thrive, difficulty with feeding despite
acid suppression or texture modifications,
aspiration/pneumonia, and cor pulmonale
(right heart failure) (Carter, Rahbar, Brigger,
Chan, Cheng, Daniel, et al., 2016). Formal
swallowing assessment should be consid-
ered if there is cough with feeding, choking,
regurgitation, feeding difficulty, no weight
gain, failure to thrive (undernutrition), or
neurologic disease. Approximately 20%
of children with laryngomalacia will have
synchronous airway lesions in the distal tra-
chea (e.g., tracheomalacia) (Dickson, Rich-
ter, Meinzen-Derr, Rutter, Thompson,
2009). Nearly 80% of children will outgrow
the condition by 18 to 24 months of age.
Children with comorbidities such as car-
diac disease, neurologic disease, respiratory
disease, or craniofacial dysmorphism are at
high risk to fail conservative management.
The children who need surgical correction
are those who have obstructive sleep apnea,
pectus excavatum, and dysphagia with or
without failure to thrive. The latter is sec-
ondary either to the poor coordination of
sucking, swallowing, and breathing and/or
the increased metabolic demand of breath-
ing that leads to poor weight gain.
Supraglottoplasty with bronchoscopy
is the standard surgical treatment (Fig-
ure 4–18). Children who are neurologically
intact without comorbidities do well with
improved stridor and breathing. While a
majority of children will improve with oral
intake, a small portion of these children
may have transient dysphagia following
supraglottoplasty that typically self-resolves
within 6 weeks (Chun, Wittkopf, Sulman,
Arvedson, 2014, 2015; Eustaquio, Lee,
Digoy, 2011).
In children who are syndromic or with
neurologic deficits, supraglottoplasty is still
quite successful (67%). Some of these chil-
dren who fail supraglottoplasty may need
tracheostomy (13%) or G tube (7%) (Dur-
vasula, Lawson, Bower, Richter, 2014).
If there are persistent signs/symptoms
following supraglottoplasty, formal evalu-
ation for extra-esophageal reflux (EER),
a brain MRI to evaluate for neurologic dis-
ease if present, or a sleep study to evaluate
for obstruction may be needed. Some chil-
dren with refractory disease and symptoms
may need tracheostomy and/or gastrostomy
tube feeding.
Laryngeal Cleft (LC)
Congenital laryngeal or laryngotracheal cleft
(LC) is an embryologic failure of complete
formation of the posterior laryngotracheo-
esophageal septum with resulting incom-
plete separation of the laryngotrachea and
pharyngoesophagus. The resulting defect
allows for an abnormal communication
between the laryngotracheal airway and
upper GI tract.
Laryngeal cleft is a rare disorder with an
incidence of approximately 1 in 10,000 live
births, although it has been reported with
increasing frequency over the past decade
likely due to increased awareness of both
laryngeal clefts and pediatric dysphagia pre-
sentations. The incidence of laryngeal cleft in
patients with aerodigestive disease has been
reported at 4.4% (Ojha, Ashland, Hersh,
Ramikrishna, Maurer, Hartnick, 2014).
Types and Classification of Laryngeal
Clefts. As described by Benjamin and
Inglis (1989), laryngeal clefts range in their
cranial-caudal depth from the type 1 supra-

cricoid defect, isolated to the laryngeal
airway, to defects that extend into the cri-
coid, cervical, or thoracic trachea (types 2
through 4, respectively) (Benjamin Inglis,
1989) (Figure 4–19). Additionally, submu-
cous clefts of the posterior larynx have been
described as a type 0 laryngeal cleft.
Syndromic associations are reported
including Optiz G/BBB syndrome, Pallister
Hall syndrome, VACTERL association, and
22q11 monosomy (including CATCH -22
and DiGeorge syndrome). The majority of
laryngeal clefts are sporadic and the index
of suspicion for laryngeal cleft pathology
should be increased when other midline
anomalies are present. The co-incidence of
second congenital anomalies is reported as
16% to 68% with a predominance for anom-
alies of the GI and respiratory tracts (Rahbar
et al., 2006). Midline anomalies are common
Figure 4–18. A. Severe laryngomalacia requiring supraglot-
toplasty. Note stretched and tight aryepiglottic fold. B. Re-
leased aryepiglottic fold.
B
A

including cardiovascular, GI, and urologic
anomalies. In patients with laryngeal clefts,
esophageal atresia and tracheoesophageal
fistula have been reported in 20% to 37% of
patients (Evans, Courteney-Harris, Bailey,
Evans, Parsons, 1995; Mahour, Cohen,
Woolley, 1973). Similarly, in a series of
139 patients with tracheoesophageal fistula/
esophageal atresia, approximately 25% of
patients had a concomitant laryngeal cleft
(Hseu et al., 2015).
Signs and symptoms of laryngeal cleft
pathology vary with approximately 50%
presenting with swallowing deficits, 37%
with respiratory symptoms, and 47% with
laryngeal or pharyngeal symptoms such
as voice disturbance of pharyngeal hyper-
secretion (Adil, Gergin, Kawai, Rahbar,
Watters, 2016; Pezzettigotta, Leboulanger,
Roger, Denoyelle, Garabedian, 2008; Rah-
bar et al., 2006).
Signs and Symptoms of Laryngeal
Clefts. Signs/symptoms related to laryn-
geal clefts reflect the depth of the defect.
n Type 1 laryngeal clefts present with
significant variability, and symptoms
are related to the effects of laryngeal
penetration or aspiration (Figure 4–20).
In general, type 1 laryngeal clefts may be
silent or present with mild to moderate
Figure 4–19. Benjamin and Inglis’ original classification. Type I: supraglottic, interarytenoid
cleft, above the vocal fold level. Type II: cleft extending below the vocal folds into the cricoid
cartilage.Type III: cleft extending through the cricoid cartilage and into the cervical trachea.Type
IV: cleft extending into the thoracic trachea, potentially down to the carina. (Source: Reprinted
with permission from Benjamin, B., Inglis, A. [1989]. Minor congenital laryngeal clefts: Diag-
nosis and classification. Annals of Otology, Rhinology, and Laryngology, 98(6), 417–420.
doi:10.1177/000348948909800603)

175
Figure 4–20. Type 1 laryngeal cleft. A. Pre-injection. B. Dur-
ing injection. continues
B
A

signs/symptoms. Typically diagnosis is
not suspected until well after birth. Clas-
sically, patients present with dysphagia
to thin liquids, chronic cough or throat
clearing, or pulmonary symptoms that
include asthma, wheezing, reactive
airway disease, and recurrent pulmonary
infections including recurrent aspiration
pneumonias, although the latter is more
commonly associated with deeper clefts.
n Type 2 laryngeal clefts tend to present
earlier in life than the type 1 clefts, with
moderate to severe respiratory symp-
toms associated with feeding. When
presenting later, or diagnosis has been
delayed, signs may include swallowing
and feeding disorders and dysphonia,
as well as chronic daily pulmonary
symptoms and recurrent aspiration
pneumonia.
n Types 3 and 4 laryngeal clefts usually
present at birth. Symptoms are typically
severe and include airway obstruction
secondary to prolapse of esophageal
mucosa into the tracheal airway, and
feeding-related symptoms mimicking
the presentation of tracheoesophageal
fistula. The severity and prognosis are
associated with depth of extension. The
involvement of the distal trachea and
carina is associated with the poorest
prognosis (Mathur, Peek, Bailey,
Elliott, 2006).
Diagnosis of Laryngeal Cleft. While
symptoms may suggest laryngeal cleft pa-
thology, definitive diagnosis of a laryngotra-
cheal cleft requires surgical endoscopy and
detailed three-dimensional examination
and palpation of the laryngotracheoesoph-
ageal complex under anesthesia (Johnson,
Watters, Ferrari, Rahbar, 2014).
Ancillary studies such as a VFSS or
FEES examination may prove helpful.
C. Post-injection.
C

Posterior laryngeal penetration or aspi-
ration at the level of the glottis in lateral
view on VFSS, or posterior interarytenoid
penetration or aspiration noted on FEES
exam increases the index of suspicion for
an underlying laryngeal cleft. The decision
to proceed with VFSS should balance the
risks of radiation exposure with the poten-
tial diagnostic benefit and may prove most
useful in patients without clinical signs of
dysphagia where the outcome of a study
may influence decisions to proceed with
further evaluation under anesthesia. Hersh
and colleagues (2016) reviewed 78 children
with type 1 laryngeal clefts. They noted that
patients averaged 3.24 VFSS each averaging
0.16 mmSv, or the equivalent of 9.4 pediat-
ric chest radiographs per study (Hersh et al.,
2016). A follow-up report suggested defer-
ring postoperative VFSS in patients with
clinical signs of dysphagia in favor of clini-
cal monitoring, and delaying postoperative
VFSS in patients with significant comor-
bidities to allow sufficient time for heal-
ing (Wentland et al., 2016). In some cases,
deferring preoperative VFSS may be con-
sidered in patients with clinical symptoms
of dysphagia or aspiration and who are
undergoing diagnostic surgical endoscopy
regardless of VFSS outcome, VFSS may be
reconsidered if a laryngeal cleft is not iden-
tified or if symptoms fail to improve post
intervention.
Treatment of Laryngeal Cleft. Treatment
of types 2–4 laryngotracheal clefts involves
endoscopic or open surgical closure of the
posterior laryngotracheoesophageal defect.
While types 1–3 laryngeal clefts are amena-
ble to endoscopic repair, most type 4 laryn-
gotracheoesophageal clefts will require an
open surgical procedure (Figures 4–21 and
4–22). It is noted that conservative therapy,
including dietary modifications, GER phar-
macotherapy, and feeding therapy may be
appropriate in some patients with type 1
laryngeal clefts. A consensus statement by
the International Pediatric Otolaryngology
Group advocated for 3 to 12 month trial of
conservative therapy prior to consideration
of surgical intervention. However, 75% to
80% of these patients did not experience an
effective response to conservative therapy
(Yeung et al., 2017).
Advocacy for conservative therapy ex-
ists, in part, due to controversy over the
degree of pathology that may be attributed
to submucosal or type 1 laryngeal clefts.
In recent years, injectable hyaluronic acid
gel, methylcarboxycellulose gel, or Gelfoam
matrix has been increasingly used at time
of initial diagnosis to fill temporarily or
efface the interarytenoid defect. Improve-
ment in symptoms or on VFSS following
injection laryngoplasty suggests the laryn-
geal cleft contributed significantly to patient
symptoms (see Figure 4–20). In a series of
68 patients treated with injection laryngo-
plasty, approximately 75% patients experi-
enced improvement or resolution of symp-
toms suggesting the laryngeal cleft was the
primary defect. Of those that subsequently
underwent endoscopic surgical repair, 90%
had experienced resolution of their dyspha-
gia (Thottam, Georg, Chi, Mehta, 2016).
Patients who do not experience significant
improvement in symptoms should be cau-
tioned that swallowing dysfunction or air-
way symptoms may likely persist if endo-
scopic surgical repair is pursued. Overall,
patients with significant comorbidities,
including cardiorespiratory disease, con-
genital syndromes, and neuromuscular
disorders tended to have prolonged recov-
eries and worse postoperative outcomes
compared with children without signifi-
cant comorbidities (Wentland et al., 2016).
In a review of 60 patients who underwent
otherwise successful surgical laryngeal cleft
repair, persistent dysphagia as determined

178
Figure 4–21. Type 2 or 3 laryngeal cleft.Top/diagonal arrow =
tracheal airway;horizontal arrows = true vocal folds;e = esopha-
geal mucosa prolapse into glottis and subglottis; a = arytenoid
joints.
Figure 4–22. With a laryngeal spreader in place exposing
the cricoid (c) and subglottis, a right angle probe is passed
between the prolapsing esophageal mucosal (e) along the
length of the cleft to determine depth. A type 2 will have a
remnant of intact cricoid cartilage at apex. Note a white tra-
cheostomy tube is seen distal to the probe. The (a) denotes
right arytenoid cartilage.

by VFSS or FEES was present in 28% of
patients with 10% remaining fully nothing
by mouth (NPO) and an additional 18%
requiring ongoing dietary modifications
(Osborn, de Alarcon, Tabangin, Miller, Cot-
ton, Rutter, 2014). This study found that
neurologic impairment and baseline gas-
trostomy tubes were predictors of the need
for NPO status. Duration of NPO status is
likely to vary considerably from one child
to another.
Sleep Disturbances
Sleep disturbances include a wide variety of
sleep disorders (e.g., obstructive sleep apnea
and frequent night awakenings). Obstruc-
tive sleep apnea is defined as absence of
airflow for at least two respiratory cycles
with persistent thoracic effort during apnea
events. In the pediatric population with no
neurologic deficits, obstructive sleep apnea
presents in both infants and young chil-
dren and is almost always accompanied by
hyperplasia of the nasopharyngeal adenoid,
palatine tonsils, or lingual tonsil. Young
infants and children with swallowing and
feeding difficulties may present with failure
to thrive/undernutrition. Decreased caloric
intake and increased caloric demands from
increased work of breathing during sleep
may both be operant. Alterations in growth
hormone secretion during sleep may also
be responsible (Goldstein, Wu, Thorpy,
Shprintzen, Marion, Saenger, 1987).
Older children may present with dys-
phagia, more likely with solid food than
liquids, although data are needed. Tonsil-
lectomy, adenoidectomy, or both will almost
always relieve the obstruction, except in
those instances when accompanying neu-
rologic compromise is present. In children
with dysphagia who do not have neuro-
logic deficits, tonsillectomy demonstrated
improvement in swallowing-related quality
of life, ability to tolerate a regular diet, and
weight percentile for age (Clayburgh, Mil-
czuk, Gorsek, Sinden, Bowman, MacAr-
thur, 2011).In children with neurologic
impairment and dysphagia, tonsillectomy
may improve swallowing in a significant
proportion of these children. However,
postoperatively there is a perioperative
risk of aspiration and possible new onset
of aspiration on swallow studies (Conley
et al., 2009). Pharyngeal and hypopharyn-
geal muscular hypotonia may then have a
significant role. Enlargement of the lingual
tonsil and swelling of the soft palate may
present with obstructive sleep apnea and
odynophagia and dysphagia. When airway
obstruction does not respond to appropri-
ate medical therapy or tonsillectomy and
adenoidectomy, alternative treatments to
be considered include continuous positive
oral or nasal pressure at night or in some
instances tracheostomy.
Children with recurrent, very frequent
(greater than 8–10) nocturnal arousals often
wake up irritable and demand a drink. Day-
time eating patterns are often erratic. Weight
gain after adenotonsillectomy occurs primar-
ily in patients who are smaller and younger
at the time of surgery and does not correlate
with increased rates of obesity (Czechow-
icz Chang, 2014). Prospective studies are
needed to elucidate the relationship of weight
gain following tonsillectomy.
Tracheostomy and Swallowing
Tracheostomyisasurgicalprocedureresulting
in the formation of a direct passage between
the trachea and skin to provide an alternate
pathway for respiration (Figure 4–23). The
surgical opening is maintained by a trache-
ostomy tube, which requires constant care
and monitoring in order to prevent serious

complications, some of which can result in
death (Rosingh Peek, 1999).
Tracheostomies are performed for a vari-
ety of conditions in the pediatric population.
These conditions most often include upper
airway obstruction, neurologic impairment,
chronic aspiration, and chronic pulmonary
disease. When tracheostomy is recom-
mended for chronic aspiration, the problem
of the aspiration is seldom solved and may
even be made worse. However, some believe
that management of tracheal secretions may
improve because of improved access for
suctioning. Short-term tracheostomy with
cessation of oral feeds in select patients with
aspiration may prove beneficial depending
on overall status and prognosis. In contrast,
when tracheostomies are placed for subglot-
tic stenosis, some patients may be able to
swallow without problems.
Swallowing problems are ubiquitous
in the pediatric population with trache-
ostomy, particularly because an increased
number of these patients have multiple dis-
abilities (DeMauro et al., 2014; Mammel,
2014; Overman et al., 2013). The pharyn-
geal phase of swallowing is most affected
by the presence of a tracheostomy tube. As
described in Chapter 2, during a normal
swallow, the bolus passes through the phar-
ynx and hyolaryngeal excursion occurs as
initiation of a swallow occurs. Alterations
in swallowing efficiency, particularly a
delayed swallow initiation in children with
tracheostomy, have been described (Abra-
ham Wolf, 2000). Mechanical fixation
of the larynx in the neck by the tracheos-
tomy tube prevents superior excursion of
the entire larynx, especially the arytenoids
and epiglottis. Closure of the laryngeal ves-
tibule is then delayed, sometimes resulting
in laryngeal penetration. Laryngeal closure
may be delayed until after opening of the
upper esophageal sphincter. Furthermore,
in adults and some older children, in whom
a cuffed tracheostomy tube is required for
tight seal of the airway to maintain ven-
tilation, cuff pressure transmitted to the
esophagus may also interfere with swallow-
ing. When cuffed tracheostomy tubes are
deflated, secretions and food pooled above
the cuff may enter into the lower airway.
Partial deflation of cuffed tracheostomy
tubes and potential for oral feeding are dis-
cussed in Chapter 9.
The cough reflex is often blunted or
absent in patients with tracheostomy. Diver-
sion of air through the tracheostomy may
Figure 4–23. A. A tracheotomy. B. A tracheostomy tube in an infant’s neck.
A B

further desensitize the larynx and result
in a blunted cough reflex (Sasaki, Suzuki,
Horiuchi, Kirchner, 1979). The inability
to generate adequate intrathoracic pressure
is the major contributor. Fitting patients
with a Passy-Muir speaking valve increases
subglottic pressure and has proven to be
most effective in improving cough and pul-
monary function in these patients (Eibling
Gross, 1996). Whereas speaking valves
have been shown to decrease laryngeal pen-
etration and aspiration in tracheostomized
adults, comparable benefits have not been
seen in children, although, pyriform sinus
residue decreased (Ongkasuwan et al., 2014).
A weak or absent cough in children is associ-
ated with increased risk of failing extubation
and need for tracheostomy in some patients
in neurocritical care units (Cohn et al., 2018).
Case Studies
Case Study 1
Presentation
Susie presented to the Pediatric Otolaryn-
gology Clinic at a Children’s Hospital at
chronologic age 3 months (corrected age
about 1½ months) with her foster mother.
Primary concern related to prominent
upper airway noises and difficulties coor-
dinating sucking, swallowing, and breathing
for bottle-feeding, which had been noted
since birth.
History
Little was known about her prenatal and
birth history, except that she was born at 34
weeks’ gestation with birth weight of 1690 g
(3 lb 12 oz; 14%ile) which was described as
“low birth weight” for gestational age. She
spent 3 weeks in a neonatal intensive care
unit (NICU) and was discharged to foster
care as a total oral feeder.
Diagnostic Workup
Diagnosis of severe laryngomalacia was
made per examination with flexible naso-
pharyngoscopy. Her interval history was
negative for pulmonary infections, wheez-
ing, or use of respiratory or reflux medica-
tions. During that clinic visit, her physical
examination was notable for mild brachio-
cephaly and torticollis, but negative for
retrognathia/micrognathia, cleft palate, or
craniofacial dysmorphism. Vocal quality
during cry was normal, Susie was noted to
have both supra- and substernal retractions
with inspiratory stridor prominent during
and apart from feeding. The foster mother
reported frequent inspiratory stridor when
Susie is asleep.
Findings Related to
Swallowing Function
Discussion for follow-up included a need
for additional delineation of Susie’s swal-
lowing function. Susie was taking 1.5 to
2 ounces EnfaCare formula per feeding
over about 20 to 30 minutes with frequent
struggles to coordinate sucking, swallowing,
and breathing, per foster mother. She had
small split-ups after each feeding, and slow
weight gain and failure to thrive (undernu-
trition) were of concern. Discussion was
held regarding likely possibility of need for
a surgical procedure—supraglottoplasty,
given the severity and frequency of inspira-
tory stridor events.
VFSS was completed 6 days following
the ENT clinic appointment. She was noted
to have inspiratory stridor as well as stertor,
with mouth frequently open as though she
was doing a combination of mouth and nose

breathing. She did not appear to have open
mouth posture due to hypotonia which may
be the case in some young infants. Findings
revealed no aspiration, no laryngeal pene-
tration, and no residue in the pharynx with
any swallows of thin liquid via wide-based
nipple used at home and a standard-type
nipple as she was positioned semi-upright
with good support to maintain trunk, neck,
and head midline. Nasopharyngeal back-
flow was noted occasionally just to the
superior side of the soft palate, which she
cleared with the next swallow. Suck:swallow
ratio ranged from 1:1 to 2:1, which is con-
sidered functional. Thus, she should not be
at major risk for aspiration concerns while
continuing to feed by bottle/nipple, with
some adjustments that included external
pacing and close monitoring of flow rate
and signs of stress.
Recommendations
and Management
Primary recommendations included the
following: (a) continue bottle-feeding per
guidelines to foster mother; and (b) return
to otolaryngologist for follow-up regarding
severe laryngomalacia.
Note: All professionals must keep in
mind that a stable airway is a prerequi-
site for oral feeding. The work of feeding
typically increases the work of breathing.
Breathing always takes priority. Thus, deci-
sions for management of the airway must be
carried out first. In addition, the interface
between spitting/reflux, and breathing and
weight gain needs to be monitored. So what
is next for Susie?
Supraglottoplasty was carried out within
1 week following the VFSS. Foster mother
was following through per guidelines
regarding facilitation of efficient bottle-
feeding. At 7 months Susie returned to the
otolaryngologist with a new foster fam-
ily. She was taking 6 to 8 oz per feeding in
about 20 minutes with weight gain of 20
grams per day during the 81 days between
appointments with the otolaryngologist.
At 7 months chronologic age (corrected
at 5½ months), Susie was growing well.
She had gone from 0.3% to 2.45% on the
growth chart in that interval. She was still
showing intermittent mild stridor with
occasional mild retractions. Spit-ups were
rare and not accompanied by any breath-
ing problems. Susie was making global
developmental gains as noted by “sitting
up, grabbing things and rolling over a little
bit,” per report from otolaryngologist. Note
that instability of a patient’s home life, such
as changing foster care settings, complicate
a child’s care, potentially delaying care and
making it more difficult for providers to get
the “whole picture” regarding procedures
and outcomes in those intervals.
Comment
As is often the case, these infants tend to
have multiple factors that can affect devel-
opment as well as feeding. Susie was fol-
lowed in the Craniofacial Center by a plas-
tic surgeon for her history of deformational
plagiocephaly and brachycephaly that was
described as “mild” and thus no helmet
was needed. She received physical therapy
for the congenital torticollis, which resolved
over the next several months.
Susie was seen by her pediatrician within
a few days of the otolaryngology visit. She
was gaining weight appropriately. There
were no pulmonary problems. She was
beginning to experience spoon-feeding of
thin smooth purees. She was making neuro-
developmental gains (e.g., sitting indepen-
dently, rolling over, and reaching for objects
with smiling and laughing). She is expected
to continue to make gains without need for
prolonged intervention.

Infants and children who demonstrate
any signs and/or symptoms of upper air-
way obstruction, such as stridor, stertor, or
voice changes, must be examined by phy-
sicians, most likely pediatric otolaryngolo-
gists. Feeding evaluations may need to be
postponed in some instances. However, for
other infants, short-term guidelines may
be provided for the safest possible feeding.
Once the airway is stable, providing there
are no other systems involved, parents are
anticipated to continue to follow through
to optimize efficiency and safety of devel-
opmentally appropriate feeding. No further
follow-up should be needed, unless par-
ents determine a need for additional guid-
ance to facilitate expansion of textures in
Susie’s diet.
Case Study 2
History
“Liam” was born at 36 weeks’ gestation via
normalvaginaldelivery.Birthweightwas3.2
kg. At birth, an orogastric (OG) tube could
not be passed into the stomach. Abdominal
x-ray showed the OG tube at the level of
the clavicles and the stomach filled with air.
A proximal esophageal atresia with distal
tracheoesophageal fistula was repaired (see
Figure 4–12A). Postoperatively, the infant
had some difficulty feeding as well as stri-
dor and a weak voice. The hospitalization
was complicated by the following: treatment
for sepsis after prolonged rupture of mem-
branes; seizures; Grade II intraventricular
hemorrhage; venular malformation (some-
times called port wine stain) of shoulder,
arm, and chest; peripheral pulmonic ste-
nosis; and coagulopathy. After 1 month,
Liam weighed 1.82 kg and was discharged
to home. Breastfeeding was supplemented
with 27 kcal formula by bottle as needed.
Examinations by Otolaryngologist
Otolaryngologic follow-up 1 week after
discharge (age about 5½ weeks) revealed
continued stridor that had worsened in the
previous week. Parents described “breath-
holding spells” between feeds and particu-
larly when he was crying. The infant became
agitated, tense, and then stopped breathing
for 20 to 25 seconds. He would gasp and
then recover. No vomiting, excessive burp-
ing, sour breath, or cyanosis was reported.
Because of the continued stridor, hoarse-
ness, and complex medical history, Liam
underwent FFNL in the outpatient clinic,
and thereafter, direct laryngoscopy, bron-
choscopy, and esophagoscopy in the oper-
ating room. On FFNL, a left vocal fold
paralysis was noted; severe reflux laryngi-
tis including mild subglottic inflammation
with narrowing also was seen. Mild tracheo-
malacia at the TEF repair site was visualized
during bronchoscopy and esophagoscopy.
Follow-Up Complications
Requiring Intensive Care Unit
Postoperatively, this infant had experienced
continued apnea and bradycardia episodes
that required intensive care monitoring.
He required oxygen and manual ventila-
tion. A 24-hour dual-channel pH probe
showed severe GERD/EERD. Medical and
positional treatment for reflux was begun;
however, the family was advised that the
child might need a tracheostomy because
of the multiple levels of obstruction—the
supraglottic swelling, the unilateral vocal
fold paralysis, the subglottic swelling, and
the mild tracheomalacia.
Over the next 3 weeks, the apnea–
bradycardia episodes decreased. Inspiratory
stridor had diminished, and Liam’s voice
became much stronger. Repeat FFNL re-
vealed a left vocal fold paresis and markedly

diminished laryngeal swelling. Mother noted
thatLiamwascalmerandatewithoutbecom-
ing agitated or tense. She continued to breast-
feed as a primary means for meeting nutri-
tion/hydration goals. Formula supplements
were added as needed. Liam was discharged
weighing 3.2 kg with expectation that he
would continue to be a total oral feeder with
appropriate growth and development.
Comment
TEF repair sometimes results in tempo-
rary or permanent vocal fold paralysis. In
this case, mediastinal edema was the most
likely cause of the paralysis that began to
improve with time, indicating a reversible
injury. Tracheomalacia to some degree is
ubiquitous after TEF repair, as is gastro-
esophageal reflux. The vocal fold paralysis,
laryngeal edema, and mild tracheomalacia
all contributed to the stridor. In this case,
the gastroesophageal reflux became patho-
logic and caused GERD/EERD. Once the
laryngeal edema (supraglottic and subglot-
tic) was treated, the stridor subsided.
The breath-holding spells with apnea
and bradycardia were also due to GERD/
EERD. consistent with one or more of the
mechanisms previously described. Liam is
now thriving, and the family is pleased with
his eating, breathing, and growing. He will
undergo laser treatments for his venular
malformation. His GERD/EERD and air-
way status will also be followed regularly to
identify relapse early if it occurs.
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191
5Pediatric
Gastroenterology
Ellen L. Blank
Introduction
Gastrointestinal (GI) disorders in children
are commonly encountered in conjunction
with swallowing and feeding difficulties.
A functional digestive system is necessary
to allow children to consume and digest
food, grow optimally, and acquire the social
and developmental skills that follow from
sharing a meal with their families. Pediat-
ric gastroenterologists possess combined
knowledge of the anatomy and physiology
of the gut, its interactions with other organ
systems, and nutrition. They have the abil-
ity to perform diagnostic and minimally
invasive therapeutic procedures to treat
certain aspects of feeding disorders. These
skills expand abilities to achieve success in
feeding either by restoring an intact gut to
normal function or by optimizing the func-
tion of a digestive system that is affected by
either a congenital anomaly or some other
alteration after birth.
This chapter covers a variety of topics
related to GI factors that impact swallow-
ing and feeding. An understanding of the
normal anatomy and physiology of swal-
lowing is covered in Chapter 2, and assess-
ment and treatment of the nutritional needs
of children with feeding disorders are found
in Chapter 6. In this chapter, delivery of
nonoral feedings is discussed, followed by
a review of gastroesophageal (GE) reflux,
and common GI etiologies as well as their
potential association with feeding difficul-
ties that develop during childhood. Case
studies at the end of the chapter provide
examples of clinical applications.
Methods of Delivery
of Feedings
Infants and children with swallowing and
feeding problems often require alterna-
tive methods of feeding to obtain adequate
nutrients and fluids for normal growth and
development. Infants and children with
congenital metabolic disorders may have
normal sensorimotor skills but refuse to eat
unpalatable defined metabolic diets. Other
children with excessive fluid needs may be
able to manage daily caloric, but not fluid,
requirements to thrive and grow. If adjust-
ments in breast- or bottle-feeding practices
for infants do not result in adequate oral
intake, other options must be considered
(Chapters 6 and 9). The two options for
nonoral supplements available are enteral
feedings that use the GI tract for digestion
and absorption, and parenteral feedings that
bypass the GI tract and provide nutrients

directly to the bloodstream. Enteral feed-
ings, when feasible and effective, are always
preferred. The types of enteral and paren-
teral feeds, with their advantages and dis-
advantages, are found in Table 5–1.
Enteral Nonoral
Feeding Methods
Naso-/Orogastric Tube Feeding
Enteral feedings by tube are usually the ini-
tial approach in infants and children who
have either an inability to coordinate the
suck and swallow sequence and feed orally
or who have excessive or unusual nutrient
requirements that preclude the use of oral
feedings alone. The most direct and simple
way to provide enteral feedings is by an oro-
gastric (OG) or nasogastric (NG) tube. OG
feedings are preferred by some clinicians for
young infants. Young infants are primarily
nose breathers, and care must be taken to
prevent partial functional obstruction of
the nasal airway. If a sample of acidic gastric
fluid (pH 4.0) cannot be aspirated after
placement of the feeding tube, the loca-
tion of OG or NG tube placement should
be confirmed radiologically before initiat-
Table 5–1. Advantages and Disadvantages of Alternate Routes for Feeding
Route Advantages Disadvantages
Orogastric • Good for neonates/infants
• Uses mouth instead of tiny
nares
• Preserves GI tract function
• Interferes with sucking
• Poorly tolerated in older
children
• Easily obstructed or dislodged
Nasogastric • Better for older infants/children
• Interferes with infant’s nasal
breathing
• Uncomfortable
• Easily obstructed or dislodged
• X-ray or acidic pH test to verify
placement
Gastrostomy Tube
(PEG or surgical)
• Comfortable
• Hidden
• Requires GI/surgical placement
• May obstruct or dislodge
• Complications (see text)
Jejunostomy • Comfortable
• Hidden
• Requires surgical placement
• May obstruct or dislodge
• Slow continuous feedings
• Complications (see text)
Parenteral • Lifesaving when GI tract is not
functioning
• Allows directed nutrient therapy
with concentrated fluids
• Surgical procedure to place
long-term sterile catheter
• Infections and catheter-related
complications (see text)
Note. GI = gastrointestinal; PEG = percutaneous endoscopic gastrostomy.

5. Pediatric Gastroenterology 193
ing feedings to avoid accidental intubation
of the trachea. OG or NG feedings can be
provided by continuous infusion or inter-
mittent bolus feedings. Although intermit-
tent bolus feedings more nearly simulate
a normal oral feeding pattern, continuous
infusion feedings may be better tolerated
in some infants with chronic tachypnea
from cardiac or pulmonary disease, gas-
troesophageal reflux or delayed gastric
emptying, short gut syndrome, or other
conditions requiring a slower rate of presen-
tation of nutrients for digestion or intestinal
absorption.
For patients who are at risk to pull out
a naso-enteral feeding tube, a plastic bridle
may be placed around the nasal septum to
secure the feeding tube as shown in Fig-
ure 5–1. Patient selection is limited to infants
and children whose nasal passages are large
enough to accommodate both the nasogas-
tric tube in one nostril and the bridle cath-
eter in both nostrils. Young babies who are
still obligate nose breathers are not candi-
dates for use of a bridle. After passage of the
bridle catheter, the ends are clipped together
outside the nose to secure the feeding tube.
Bridles can be placed on tubes ranging from
5 to 18 French in diameter. The procedure
can be completed within a few minutes by
a trained professional without patient seda-
tion. Sutures or adhesive tape dressing are
not needed to maintain tube placement. As
with placement of any other naso-enteral
feeding tube, an x-ray should be used to
verify the internal location of the feeding
tube before feeding the patient. The bridle
should be changed monthly to alternate
nostril placement of the nasogastric tube
as with other indwelling naso-enteral tubes
(Applied Medical Technology, 2009).
If gastric feedings are poorly tolerated,
it is possible to feed directly into the duo-
denum or jejunum. While lowering the risk
of gastroesophageal reflux, aspiration, and
distension, tube placement under fluoros-
copy or by endoscopy is needed to ensure
proper placement. Coughing, retching, or
vomiting may result in displacement of the
tip of the tube proximally into the stomach,
Figure 5–1. Patient with a bridle to secure a naso-enteral
tube in place. (Source: Printed with permission from Applied
Medical Technology.)

esophagus, trachea, or even the mouth. Each
dislodge
ment requires an appointment to
an endoscopy suite or radiology depart-
ment with exposure to radiation for tube
replacement. Duodenal and jejunal tube
feedings must be given as a slow continuous
infusion. Rapid infusion of a high glucose
and solute load into the small intestine
results in postprandial GI and vasomo-
tor complaints called dumping syndrome.
Typical symptoms in pediatric patients may
include crampy abdominal pain, nausea,
retching or vomiting, explosive diarrhea,
sweating, flushing, dizziness, palpitations,
or lethargy.
Gastrostomy Tube Feeding
If long-term (greater than 1 to 3 months)
enteral feedings are required, a gastros-
tomy tube (GT) should be considered. The
development and widespread use of gas-
trostomy tubes has made long-term deliv-
ery of enteral feedings a feasible alternative
for children who cannot or will not meet
nutritional needs orally. They are particu-
larly useful when anatomic restrictions,
developmental delay, or increased needs
occur. In infants and children with aspira-
tion from oral feeding, severe developmen-
tal delay, or inadequate suck and swallow
due to a chronic condition, GT placement
early in the medical course is generally rec-
ommended. Each case must be decided on
an individual basis. Most caregivers find GT
feedings much preferable to NG feedings for
home use because of ease of care, patient
comfort, and elimination of frequent NG
tube changes and use of adhesive tape to
secure tube placement. Gastrostomy tubes
(example in Figure 5–2) can be hidden
under clothing, are not uncomfortable, and
require less skill in care. In select cases, a
gastrostomy tube can be placed percutane-
ously via endoscopy, thereby reducing the
need for a laparotomy.
Percutaneous endoscopic gastrostomy
(PEG) placement offers an attractive alter-
native to the open intra-abdominal tech-
nique. PEG may not require general anes-
thesia and can be performed safely in the
endoscopy suite for most children, includ-
Figure 5–2. Child with gastrostomy tube (button) in place. Note
placement and scar on chest from prior cardiac surgery.

ing small or young infants. In children, both
adequate sedation and control of the airway
are important during the procedure. The
patient must lie still during the PEG place-
ment. The endoscopist must be familiar with
the child and judge how much sedation will
be needed to complete the procedure safely.
In any case, adequate personnel, monitor-
ing, and equipment should be available for
patient safety during the procedure. After
adequate sedation is achieved, inability to
clearly trans-illuminate the stomach percu-
taneously because of overlying colon, liver,
spleen, or ribs results in termination of the
percutaneous procedure to avoid accidental
perforation of an adjacent organ. Although
a laparoscopic or open gastrostomy pro-
cedure imposes additional costs, safety
and successful surgical gastrostomy place-
ment certainly outweigh cost. Figure 5–3
highlights the endoscopic features of PEG
placement. After the gastrostomy stoma has
healed, caregivers can be trained to change
a balloon gastrostomy feeding tube at home
about every 3 months.
After a gastrostomy tract has healed,
an acute situation, such as pneumonia or
orthopedic traction, may arise where pre-
vention of aspiration by feeding directly
into the jejunum may be safer. Then, a gas-
trostomy tube can be replaced by a longer
gastro-jejunal (GJ) feeding tube. This type
of tube can be placed endoscopically or
radiologically through the existing stoma.
It offers both a gastric port for giving medi-
cations or venting the stomach and a jejunal
port for constant infusion of nutrition and
fluids. These tubes are changed about every
3 months.
Jejunostomy Tube
Some patients may need placement of a
feeding tube directly into the jejunum to
succeed with enteral feeding. A jejunostomy
tube is not usually the first tube placed for
feedings, but it may be necessary for patients
with recurrent vomiting or aspiration
pneumonia after gastrostomy placement,
dysmotility syndromes with poor gastric
emptying or gastric bloating, microgastria,
inability to maintain placement of GJ tube
at the gastrostomy site, or lack of nearby
radiological services to replace GJ tubes.
There are three possible ways to place a
jejunostomy tube:
n The simplest method is direct place-
ment endoscopically through the
skin into the jejunum, similar to the
procedure to place a gastrostomy in the
stomach.
n If the tube cannot be placed endos
copically, a laparoscopic or open
surgical procedure can be performed.
Once the tract has healed, a variety of
feeding tubes or button feeding tubes
can be used in the stoma. Typically, the
tubes are changed by a radiologist who
can verify that the replacement tube is
correctly positioned.
n Another method is a Roux-en-Y proce-
dure. A surgeon uses a short segment of
jejunum to create a connection, or limb,
between the jejunum and the skin. The
limb creates a more stable tract for the
feeding tube. After the tract has healed,
the patient’s caregivers can change the
balloon button or feeding tube them-
selves at home.
Complications of Tube Feedings
As with any medical/surgical procedure,
there are potential complications. Goldin
et al. (2016) studied a retrospective cohort
of 15,642 children, all with at least one com-
plex chronic condition, for complications
after undergoing gastrostomy tube place-
ment at children’s hospitals in the United

196
Figure 5–3. A. Photographs taken during percutaneous endoscopic gastrostomy
placement. Frame 2 illustrates a bulge in the stomach wall made by the endoscopy
assistant’s finger pressure on the abdomen during attempts to identify a proper loca-
tion for tube placement. Frames 6 and 7 show the placement of a snare by the endos-
copist at the site selected for tube placement. Frame 8 depicts the closure of the
snare on the needle introduced from the anterior abdominal wall into the stomach.The
guidewire coming through the needle is also identified. B. Photographs taken during
percutaneous endoscopic gastrostomy placement. Frames 9 and 11 demonstrate the
guidewire over which the gastrostomy tube is placed. Frame 12 demonstrates the
endoscopic appearance of the gastrostomy tube in place.
B
A

States. Of these patients, 8.6% returned
to the emergency department within 30
days after surgery, and 3.9% were admit-
ted to the hospital. PEG tubes amounted to
28.7% of the total, and the rest were placed
by another method. The most common
diagnoses were infection (27%), mechani-
cal complication (22%), and replacement
(19%). The authors divided complications
into two categories. Operative complica-
tions included wound infection, wound
dehiscence, and hollow viscus perforation.
Maintenance complications included tube
dislodgement and stomal infection, tube dis-
lodgement, granulation tissue, or prolapse.
They also noted that there was no differ-
ence in emergency room visits or admis-
sions between children with neurological
impairment and GE reflux who underwent
gastrostomy tube placement with or without
concomitant fundoplication.
Other operative complications of per-
cutaneously placed feeding tubes may also
occur. These complications include GI
bleeding, leakage of gastric or small intes-
tinal contents onto the skin or into the
peritoneum, esophageal laceration, colonic
perforation, gastro-colic fistula, peritonitis,
subcutaneous emphysema, external migra-
tion of the inner flange, wound infection,
peristomal excoriations, granulation tis-
sue, and symptomatic GE reflux. Esopha-
geal laceration, usually at either the upper
or lower esophageal sphincter, is apparent
at the time of a PEG placement procedure.
Primary jejunostomy permanently alters
the anatomy of the jejunum and may form
a point for future intestinal volvulus around
the Roux-en-Y limb. Colonic perforation
or gastro-colic fistula may not be evident
until the initial PEG tube is replaced with
a button tube months later. Problems with
the stoma itself that are usually visible on
the anterior abdominal skin surface lead to
prompt attention.
Nonsurgical complications of feeding
tube placement include vomiting, abdomi-
nal distention, and diarrhea. For patients
who already have gastroesophageal reflux,
more frequent vomiting after gastrostomy
tube placement warrants further investi-
gation to look for an obstruction or infec-
tion. If no other source for the vomiting is
found, a nutritionally complete formula of
relatively low osmolality may be better tol-
erated. If diarrhea persists, a more elemental
formula could be considered.
Metabolic complications of tube feed-
ings are less common than GI complications
unless the patient is severely malnourished.
Severely malnourished patients who will
need an enteral feeding tube may be par-
tially nourished before undergoing a pro-
cedure to place a permanent feeding tube.
The refeeding process includes temporary
NG tube placement, slower than normal
tube feedings with initially dilute enteral
formula, and intravenous fluid and electro-
lyte supplements as needed. Metabolic signs
indicating refeeding syndrome include
hyperamylasemia, hypokalemia, hypomag-
nesemia, hypophosphatemia, and hypocal-
cemia and are commonly monitored when
starting to nourish such patients.
Maintenance complications of enteral
feedings vary, depending on the method
of delivery. NG, OG, and GJ tubes must
be checked carefully for proper tube place-
ment. Concentrated enteral feedings, medi-
cations, and insufficient water flushes after
use of tubes commonly contribute to clog-
ging. The safest way to unclog a feeding tube
is use of warm water in a syringe and gentle,
steady push and pull on the plunger to cre-
ate agitation in the tube, and then clamping
the tube for up to 30 minutes. Allowing the
water to soak into the clog may also help to
restore patency. Use of carbonated beverages
and meat tenderizer has not been shown to
be effective (Fisher Blalock, 2014).

Parenteral Feeding
In the past, parenteral nutrition was re-
served primarily for infants or children with
severe GI tract disorders that prevented the
use of the GI tract. Parenteral nutrition has
become a standard mode of feeding for very
small premature infants and for infants or
children in need of short-term intensive
nutritional rehabilitation (Kerner Hur-
witz, 2008). The parenteral route offers the
advantage of nutritional supplement in the
presence of GI tract malfunction. If paren-
teral nutrition is needed for a relatively short
period of time (1–2 weeks), peripheral vein
access may be preferable to the use of a cen-
tral venous catheter. Placement of a periph-
eral access catheter can be done at the bed-
side.Patientswithhigher thannormal calorie
requirements, fluid restriction (requiring
highly concentrated formulations), or those
requiring long-term parenteral nutrition
require central venous access (e.g., Broviac
or Hickman catheter). The development of
a central percutaneous intravascular central
catheter (PICC line), which is placed via a
peripheral vein but fed through to a central
venous location, has improved access for
central parenteral nutrition.
Complications of parenteral nutrition
can be categorized as metabolic or techni-
cal. Metabolic complications remain a par-
ticular concern for patients receiving long-
term parenteral nutrition. Close monitoring
of laboratory values, including glucose,
serum protein, liver function tests, and elec-
trolytes, can be used to assess tolerance to
the therapy. Thus, alterations can be made
rapidly if metabolic intolerance is observed.
Technical problems are associated primar-
ily with the use of central venous catheters
for access. Problems include those that
arise at the time of insertion of the cath-
eter and problems with the long-term use
of catheters. Problems encountered at the
time of insertion of a central venous cath-
eter include pneumothorax, injury to the
vein or artery, and air embolism. Technical
complications that may arise with use of the
catheter include venous thrombosis, sepsis,
catheter dislodgement, and perforation or
leakage, sometimes with skin slough when
a peripheral vein is used. Heparin is usually
added to parenteral nutrition solutions to
prevent thrombosis. Strict aseptic technique
must be used in caring for the catheter site
and during infusion of the parenteral nutri-
tion solutions. Although parenteral nutrition
can be lifesaving for some infants, disuse
atrophy of the GI tract and direct hyperbili-
rubinemia, leading to irreversible liver dam-
age, can and should be avoided by the intro-
duction of enteral feeds as soon as possible.
Kerner (2008) and Chapter 6 of this book
provide more complete discussions of par-
enteral nutrition in the pediatric population.
Dysphagia Secondary
to GI Disease
Common categories of GI problems that
can underlie dysphagia and may even pres-
ent as dysphagia and feeding problems
include inflammatory disease, structural
abnormalities, dysmotility disorders, and
miscellaneous conditions (Table 5–2).
Dysphagia Secondary to
Inflammatory Diseases:
Presentations, Causes,
Diagnostic Testing, and
Treatment Modalities
Recent advancements in the understanding
of eosinophilic inflammation in the diges-
tive system have transformed how clinicians
diagnose and treat patients with GER, other

eosinophilic inflammatory processes, and
esophageal infections. Applications have
extended to new methods of treatment and
maintenance therapy for inflammatory
components of upper digestive infections,
structural anomalies, and dysmotility dis-
orders. This has resulted in more possibili-
ties for improved outcomes for patients with
dysphagia, feeding difficulties, and chest
pain, as well as a heightened awareness that
more than one inflammatory process may
be present in any given patient.
Reflux Disease: GERD/EERD
Definitions. GER is defined as the invol-
untary return of stomach contents into the
esophagus. It occurs in normal humans
several times during the night and day, par-
ticularly after meals, and is a normal physi-
ological process. Episodes of GER are brief,
asymptomatic, and involve only the distal
esophagus. The passage of stomach con-
tents into the pharynx, mouth, or perioral
area is called regurgitation or spitting-up.
Regurgitation is common in infants early
in life, peaks at about age 3 or 4 months,
is considered to be effortless and painless,
and resolves by age 18 months. GER in
infancy usually resolves by about age 12 or
14 months (Hegar et al., 2009; Salvatore
Vandenplas, 2016). Gastroesophageal reflux
disease (GERD) occurs when GER results
in bothersome symptoms or complications.
In contrast to GER, vomiting is force-
ful ejection of stomach contents through
Table 5–2. Gastrointestinal Problems Causing Dysphagia and
Feeding Problems
Inflammatory disorders • Gastroesophageal reflux disorders
(GER/GERD)
• Eosinophilic esophagitis (EoE)
• Eosinophilic gastroenteritis (EGE)
• Infections
Structural anomalies
(esophagus)
• Tracheoesophageal fistula (TEF)
• Esophageal atresia (EA)
• Esophageal webs/strictures
Structural anomalies
(stomach and duodenum)
• Hypertrophic pyloric stenosis
• Antral or duodenal webs
Primary proximal esophageal
dysmotility disorders
• Cricopharyngeal achalasia
• Achalasia
Primary distal esophageal
dysmotility disorders
• Achalasia
• Diffuse esophageal spasm
• Nonspecific esophageal dysmotility
Miscellaneous • Esophageal compression
(intrinsic/extrinsic)
• Constipation
• Duodenogastric reflux (DGR)

the mouth. Unlike GER, vomiting is a more
complex process with activation of recep-
tors, not necessarily found in the GI tract,
and a coordinated autonomic and voluntary
motor response. Vomiting has been regarded
as a hallmark of GER in infancy but may not
be present in conjunction with oropharyn-
geal signs and symptoms. As a child grows,
vomiting generally occurs less often.
The term extra-esophageal reflux (EER)
or laryngopharyngeal reflux (LPR) denotes
regurgitation of stomach contents through
the upper esophageal sphincter (UES) into
the pharynx, larynx, mouth, nose, parana-
sal sinuses, tracheobronchial tree, or lungs.
Extra-esophageal reflux disease (EERD)
results when the EER results in bother-
some symptoms or complications. Effects
of EERD on the upper airway are discussed
in Chapter 4.
Epidemiology. The prevalence of GERD/
EERD is not known. Attempts to determine
the prevalence encounter multiple obstacles.
The symptoms are nonspecific. Infants and
children lacking adequate communication
skills cannot articulate their complaints.
Not all parents seek medical advice for
their children. Patients are not evaluated in
a consistent manner, and many patients may
not even have access to the latest diagnos-
tic technology. But there are also distressed
parents caring for an infant who is crying
constantly and regurgitating who may con-
tact the primary care provider repeatedly
looking for advice to help them cope and
do their best as parents.
Pathophysiology. The anatomy and
physiology of the esophagus are presented
in Chapter 2. The pathophysiologic mecha-
nisms of GERD/EERD are summarized in
Table 5–3 and described in further detail in
this section. All of these factors appear to be
interrelated, and their relative importance
remains unknown at this time. The poor
correlation between esophageal histology
and reflux exposures implies that multiple
factors must play a role.
Transient relaxation of the lower esoph-
ageal sphincter (LES) occurs normally in
response to the initiation of swallowing.
Inappropriate transient lower esophageal
sphincter relaxation (TLESR) occurs briefly
and is not associated with swallowing or
esophageal peristalsis. TLESRs have been
reported in normal individuals as well as
those with GERD/EERD (Dent et al., 1980;
Orenstein, 1992). Most reflux episodes in
children occur during TLESRs. GERD/
EERD is also more likely to develop after
compromise of esophageal peristalsis and
clearance, changes in esophageal mucosal
resistance, or changes in the anatomical rela-
tionship of the LES, crura of the diaphragm,
and the angle of His. Esophageal impedance
studies with pH monitoring have demon-
strated that weakly acidic reflux (pH 4–7)
is associated with postprandial symptoms in
infants and in patients who are resistant to
acid inhibitors. Duodenogastric reflux must
occur just before weakly acidic GER events
(Salvatore Vandenplas, 2016).
Presentations and Clinical Evaluation
of GERD/EERD. During the initial evalu-
ation, the clinician must keep in mind the
myriad of signs and symptoms consis-
tent with the diagnosis of GERD/EERD.
Table 5–4 presents signs and symptoms
from regurgitation, respiratory system,
acid-related inflammation, and neurobe-
havioral factors. No further evaluation is
indicated when a healthy, thriving infant
intermittently regurgitates formula or breast
milk after feedings as long as there are no
pulmonary concerns. Patients with chronic
recurrent forceful vomiting or slowing of
weight gain should undergo more formal
evaluation to document the presence of

GER and rule out other causes of vomiting
before considering medication.
There are some warning signs in infants
that suggest that GER is not the correct
diagnosis. The warning signs include bil-
ious vomiting, GI bleeding noted in emesis
or stool, recurrent forceful vomiting, onset
of vomiting after age 6 months, failure to
Table 5–3. Pathophysiology of GERD/EERD
Underlying
Condition
Anatomic/Mechanical
Processes Physiologic Processes
GERDa
Intrathoracic esophagus
Surgical alterations (TEF
repair)
Neurologic reflex hyper- or
hyporeactivity
Increased frequency of reflux episodes
• Transient lower esophageal sphincter
relaxations (TLESRs)
• Increased abdominal pressure
• Decreased thoracic pressure
• Decreased tone in lower esophageal
sphincter
• Gastric distention (increased volume)
• Gastric dysmotility (incoordination/delay
of gastric emptying)
• Increased gastric secretion
Increased duration of reflux episodes
• Posture, position
• Deficiency of saliva
• Dysmotility of esophagus or stomach
Content of refluxate
• Acid
• Gastric enzymes
• Bacteria
• Undigested food
EERDa
Surgical alteration (TEF,
colonic interposition)
Upper airway obstructive
lesions
Decreased upper esophageal sphincter tone
(especially at night)
Impaired reflexes (e.g., esophago-laryngeal,
esophago-pulmonary)
Esophagitis
Content of refluxate
• Same as for GERD, but effect may be
greater on respiratory mucosa than on
esophageal epithelium
DGRa
Pyloric incompetence Bile salts
Pancreatic enzymes
Note. a
Most, if not all, of the pathophysiologic mechanisms for GERD are operant with EERD and DGR.
The list here includes additional effects.

202
Table 5–4. Clinical Presentation of GERD/EERD
Signs and Symptoms from Regurgitation
• Emesis (with malnutrition)
• Gagging
• Choking
• Cough
• Apnea
• Halitosis
• Frequent swallowing
• Burping
Respiratory Signs and Symptoms
• Recurrent pneumonia
• Apnea (central and obstructive)
• Blue spells
• Recurrent croup
• Stridor
• Chronic cough
• Asthma
• Hiccups
• Brief resolved unexplained events (BRUEs)
• Gurgling respirations
Signs and Symptoms from Acid-Related Inflammation
• Heartburn
• Irritability
• Food refusal
• Swallowing problems (dysphagia)
• Opisthotonus (back arching)
• Otalgia
• Torticollis (Sandifer’s syndrome)
• Chest/abdominal pain
• Hematemesis (with anemia)
• Esophageal obstruction
• Chronic laryngitis/hoarse voice
• Chronic rhinosinusitis
• Bronchospasm/laryngospasm
Neurobehavioral Signs and Symptoms
• Infant “reflux” spells (seizure-like with posturing, apnea,
cyanosis)
• Severe sleep disturbances
• Irritability
• Food refusal
Note. EERD = extra-esophageal reflux disorder; GERD = gastroesopha-
geal reflux disorder.

thrive, constipation, diarrhea, fever, leth-
argy, bulging fontanelle, seizures, abdominal
distention or tenderness, hepatosplenomeg-
aly, or known/suspected genetic or meta-
bolic syndrome (Vandenplas et al., 2009).
When any of these warning signs are pres-
ent, they may represent a complication of
GER or another disorder that could present
with vomiting or regurgitation.
It is also important to identify activities
of daily life that might contribute to signs
and symptoms of GER. These activities
include use of certain other medications,
dietary habits, lifestyle habits, and expo-
sure to environmental allergens. Tobacco
(passive or active exposure), alcohol, and
caffeine all have adverse effects on LES
pressure and GI peristalsis (Vandenplas
et al., 2009). Genetics, sleep state, vigorous
exercise after eating, milk protein and other
food allergies, overeating, lying down after
eating, late-night eating before bedtime,
obesity, and stress may all contribute to the
development of GERD in older children
(Vandenplas et al., 2009).
Diagnostic Tests for Evaluation of Chil-
dren with GERD/EERD. Several diagnos-
tic tests (Table 5–5 with advantages and
disadvantages) can be used in the evalua-
tion of GER in infants and children. Tests
must be selected based on their ability to
provide the desired information. No single
test can document the presence of GERD,
exclude other conditions, and evaluate effi-
cacy of treatment. Pediatric primary care
providers may want to confer with or refer
to a pediatric gastroenterologist to choose
the best test(s) to evaluate a patient’s symp-
toms. The contrast esophagram is a radio-
graphic study during which a child swallows
a radio-opaque inert liquid, often barium,
to identify structural abnormalities of the
upper digestive system. It is not sensitive or
specific for diagnosing GER. An esopha-
gram is not recommended as the first-line
investigation for diagnosing GERD (Light-
dale Gremse, 2013; Vandenplas et al.,
2009). Nuclear scintigraphy can document
the rate of gastric emptying of solids and liq-
uids and may detect aspiration as a result of
GER to differentiate aspiration from “above”
on saliva, food, or liquid. Combined laryn-
goscopy and bronchoscopy with bronchial
washings are useful to confirm findings that
suggest aspiration following nuclear scintig-
raphy or separate from scintigraphy. As with
esophagram, routine nuclear scintigraphy is
not recommended as a primary examina-
tion to diagnose GER (Lightdale Gremse,
2013; Vandenplas et al., 2009).
Esophagoscopy can be performed on
premature infants as small as 1 kilogram
because diameters of modern flexible
endoscopes are very small. The advan-
tage of endoscopy is the ability to visualize
the appearance of the esophageal mucosa
directly and to locate the anatomic land-
marks in the distal esophagus. Clinical prac-
tice guidelines agree that reflux esophagitis
should be defined as macroscopically visible
breaks in the mucosa at or just above the
LES. Mucosal redness alone is deemed to
be an unreliable sign of reflux esophagitis
(Lightdale Gremse, 2013; Vandenplas
et al., 2009). Endoscopic biopsies from the
proximal and distal esophagus are helpful
to identify any inflammatory or infectious
process that may be causing the presenting
signs and symptoms. GER may be present
with normal or abnormal biopsies. Blind
suction catheter has been used to collect
esophageal biopsies. This procedure is less
invasive than endoscopy, does not involve
sedation, and is less expensive. If the biopsy
samples are abnormal, they may provide
helpful information. However, direct visu-
alization of esophageal mucosa or thera-
peutic treatment is not possible with this
procedure.

204
Table 5–5. The Advantages and Disadvantages of Commonly Used Diagnostic Tests to
Evaluate and Treat GERD/EERD in Children
Test Advantages Disadvantages
Contrast esophagram Readily available
Shows anatomic
abnormalities
Shows only obvious reflux
Short test
Artificial food
Radiation exposure
Low sensitivity
Nuclear scintigraphy with
gastric emptying
Uses regular food
Longer test time (1 hour)
Estimates gastric emptying
rate
May show aspiration
High specificity
Shows any reflux (acid and
alkaline)
Does not quantitate reflux
Limited time for test
Low sensitivity
Postprandial only
Direct rigid laryngoscopy
or FFNL
Readily available
Shows airway structure
Minimally invasive
Requires otolaryngologist
Bronchoscopy with
bronchial washings
Shows airway structure
May help diagnose aspiration
or infection
Confirm aspiration via
scintigraphy
Invasive
Requires otolaryngologist
Esophagoscopy Can evaluate other conditions
with biopsy
Can treat strictures
Invasive
Low sensitivity for EERD
Suction catheter
esophageal biopsy
Abnormal biopsy diagnostic
Minimally invasive
Low sensitivity
Mucosa not seen
Not therapeutic
24-hour dual-channel
prolonged pH probe
monitoring
High sensitivity
Quantitates acid reflux
Invasive
Does not always evaluate
for nonacid reflux unless
calibrated and requested
MII-pH probe High sensitivity
Measures distance of GER
Automated analysis of test
Detects acid/nonacidic GER
Reproducibility varies
Expensive
Note. EERD = extra-esophageal reflux disorder; FFNL = flexible fiberoptic nasopharyngolaryngoscopy;
GER = gastroesophageal reflux; MII-pH probe = multichannel intraluminal impedance pH probe.

Prolonged pH probe monitoring is
considered the most sensitive indicator of
GERD. This monitoring can be done at
home. It does not require hospitalization for
the 24-hour duration. Normal values have
been established to diagnose pathological
GER in children and adults. This test can
document the frequency and duration of
acidic (pH 4) GER events. An observer
manually records meals, activity, sleep, and
signs/symptoms during the study noting the
time, duration, and patient posture. These
events can then be correlated with the pH
level documented at the same time. Fol-
lowing treatment for GER, a second study
can be completed to evaluate success after
treatment. Although this procedure has
been available for more than 30 years and
has been the best test to diagnose GER, lack
of reproducibility of results remains a draw-
back (Vandenplas et al., 2009).
Multichannel intraluminal impedance
(MII) pH monitoring is a newer technology
that is gaining popularity for the diagnosis
of GERD. This test measures movement of
liquids, solids, and gas through the esoph-
agus by measuring electrical resistance
between multiple electrodes along an intra-
luminal catheter. Acidic and nonacidic GER
can be detected. Rates of antegrade and ret-
rograde bolus movement in the esophagus
can be measured. Research is ongoing in
examination of the utility of MII combined
with a pH probe in looking for correlations
between GER events and patients’ symp-
toms (Rosen, Lord, Nurko, 2006). This
test may be useful to assess efficacy of treat-
ment after obtaining a baseline study before
treatment.
Severe Chronic GERD: Barrett’s Esoph-
agus. Patients with years of long-standing,
severe chronic GERD may develop meta-
plastic changes in the epithelium of the
distal esophagus. Once the metaplastic
changes are evident, a diagnosis of Barrett’s
esophagus can be made. It is uncommon in
children. The diagnosis is difficult to differ-
entiate from extensive untreated esophageal
inflammation and is more likely to be vis-
ible at follow-up endoscopy after at least 12
weeks of aggressive proton pump inhibitor
(PPI) treatment. Barrett’s esophagus is not
an absolute indication for antireflux surgery
unless the reflux esophagitis cannot be con-
trolled medically with aggressive continu-
ous PPI treatment. Intervals for surveillance
with endoscopic biopsies, based on expert
opinion by adult gastroenterologists, are
based on the level of dysplasia. Patients with
Barrett’s esophagus who have undergone
fundoplication merit surveillance follow-
up, too. For pediatric patients with GERD
who have not developed dysplasia, expert
opinion suggests endoscopic surveillance
by a pediatric gastroenterologist with biop-
sies every 3 to 5 years throughout adoles-
cence with a clearly planned transition to an
adult-focused gastroenterologist at adult-
hood (Shaheen, Falk, Iyer, Gerson, 2015).
Treatment Modalities for GERD/EERD
(Table 5–6)
Conservative Approaches (Nonmed
ical, Nonsurgical). Parents usually seek
medical attention when their infant between
ages 1 and 6 months has frequent regurgi-
tation and appears to be in distress. For
healthy infants, who are thriving, the first
step is a trial of nonmedical, nonsurgical
strategies such as reassurance, avoidance of
exposure to tobacco, a discussion of appro-
priate feeding volume based on the infant’s
needs, and compassion for the parental
distress from witnessing the frequent pro-
longed crying. A diary of activities and feed-
ings kept by the caregivers provides help-
ful information to offer reassurance or to
tailor recommendations to the particular

family situation. If these recommendations
are not helpful and the infant continues
to regurgitate at least four times daily for
another 2 weeks, removing cow’s milk from
the diet of mothers who are breastfeeding
or switching a formula-fed infant to either
an AR-formula (antiregurgitation formula),
partial-hydrolysate formula, or amino-acid-
based formula may be helpful (see Chapter
6). Expert opinion (Lightdale Gremse,
2013; Vandenplas et al., 2009) suggests AR-
formula rather than adding rice cereal to the
formula at home because the commercially
made formulas control the amount of thick-
ener and final osmolarity of the formula to
maintain balanced infant nutrition. Use of
an AR-formula should decrease regurgita-
tion (Horvath, Dziechciarz, Szajewska,
2008). Partial-hydrolysate formulas have
better digestibility and gastric emptying
than standard infant formulas (Shergill-
Bonner, 2010). Use of an amino-acid-based
formula is indicated when cow’s milk pro-
tein allergy is suspected or confirmed. The
use of positioning is controversial. One study
has shown that AR-formula is as effective as
upright positioning to treat infant regurgita-
tion (Chao Vandenplas, 2007).
For older children with signs and symp-
toms of regurgitation, nonmedical, nonsur-
gical recommendations may include avoid-
ance of alcohol, chocolate, spicy foods, and
caffeine if they provoke symptoms; weight
loss if overweight; no eating for at least 2
to 3 hours before bedtime, and left lateral
decubitus position for sleep with the head
of the bed elevated (Vandenplas et al., 2009).
Antacids may provide short-lived relief of
heartburn. Prolonged use of high doses of
antacids containing calcium carbonate or
aluminum may result in significant side
effects. Antacids and sucralfate, a surface-
active resin-containing aluminum, have
not been well studied in pediatrics. Expert
opinion does not recommend surface-active
agents for sole treatment for erosive esopha-
gitis (Lightdale Gremse, 2013; Vanden-
plas et al., 2009).
Pharmacologic Treatment
Acid Suppression. Acid suppression
is the preferred pharmacologic choice for
treatment of GERD in children and adults.
PPIs have been shown to be more effec-
tive than histamine-2 receptor antagonists
(H2RA) to curb symptoms even though
they do not reduce the incidence of reflux
episodes. Studies in older children gener-
ally show improvement in GER symptoms
with treatment with PPIs; however, the
same treatment in infants generally does
not improve GER symptoms (Salvatore
Vandenplas, 2016). Medications available in
the United States are included in Table 5–7.
Dosages vary by drug, a given patient’s age
and weight, and the diagnosis being treated.
Preparations of H2RA are often easier and
less expensive to administer than most for-
mulations of PPIs. The use of PPIs in infants
Table 5–6. Treatment Strategies for
GERD/EERD
Nonmedical, Nonsurgical
• Dietary
• Position
• Activity
Pharmacologic
• Antacids
• Histamine-2 receptor antagonists
(H2RAs)
• Proton pump inhibitors (PPIs)
Surgical
• Jejunostomy feeding
• Fundoplication
• Gastroesophageal separation
Note. EERD = extra-esophageal reflux disorder;
GERD = gastroesophageal reflux disorder.

207
Table 5–7. Commonly Prescribed Pharmacologic Agents for Pediatric Patients with
GERD in the United States
Medication Type
FDA
Approval
for Children
Liquid
Formulation
When to
Administer
Can be
Given by NG/
Gastrostomy
Cimetidine
(Tagamet HB)
H2RA 16 years 300 mg/5 ml With food
or antacid
Yes
Ranitidine
(Zantac)
H2RA 1 month–
16 years
15 mg/ml With meals
or bedtime
Yes
Famotidine
(Pepcid)
H2RA 1–16 years Chew tab or
powder added
to water
With or
without
food
Yes
Nizatidine
(Axid)
H2RA 12–16 years 2.5 mg/ml
made from
capsule with
Gatorade, cran-
grape or V-8
100% vegetable
juice
With or
without
food; do not
give with
apple juice
Yes
Omeprazole
(Prilosec)
PPI 1 month–
16 years
Open capsule,
add granules to
water
30 minutes
before
meals
Yes
Lansoprazole
(Prevacid)
PPI 1–16 years 15 mg oral
dissolving
tablet or open
capsule, add
granules to
puree or juice
30 minutes
before
meals
Yes
Esomeprazole
(Nexium)
PPI 1 month–
16 years
Granule packet,
add to water
or applesauce.
also capsule to
swallow
60 minutes
before
meals
Yes
Rabeprazole
(Aciphex)
PPI 1–16 years Open capsule,
granules added
to pureed
texture food
30 minutes
before
meals
Not specified
Pantoprazole
(Protonix)
PPI 5–16 years Dissolve tablet
in apple juice
30 minutes
before
meals
Yes
Note. Patients older than age 16 years are considered adults for prescribing purposes here. EERD =
extra-esophageal reflux disorder; FDA = Food and Drug Administration; GERD = gastroesophageal reflux
disorder. Adapted from Lexicomp Online®, 2017.

and children is not universally U.S. Food
and Drug Administration (FDA) approved.
In the case of infants and children who are
tube fed, lansoprazole oral dissolving tablets
are particularly convenient to administer
compared to opening a capsule and stirring
granules into puree-textured food.
Since 2002, there has been a rapid
increase in the use of PPIs in the United
States, especially in young children, to treat
symptoms assumed to be caused by GER. In
2010, lansoprazole was the 9th and raniti-
dine was the 14th most prescribed medica-
tion for children younger than 2 years old in
the United States (Chen et al., 2012). Heine,
Jaquiery, and Lubitz (1995) looked for cor-
relations of infant behavior attributed to
episodes of GER and documented episodes
of GER. They found no correlation between
documented episodes of GER and crying,
feeding refusal, slow weight gain, sleep dis-
turbances, and back-arching. There is no
evidence that sleep disturbances or crying in
infants is a manifestation of GER or that PPI
treatment will decrease the signs or symp-
toms. Placebo-controlled prospective stud-
ies of PPI have found no evidence that sleep
disturbances or crying in infants is a mani-
festation of GER or that PPIs will decrease
the symptoms. Some patients treated with
PPIs had adverse effects from the medica-
tion. Idiosyncratic reactions (Vandenplas
et al., 2009), increased susceptibility to
community-acquired respiratory infec-
tions (Giuliano, Wilhelm, Kale-Pradhan,
2012), and changes in GI flora or bacterial
overgrowth (Hegar, Hutapea, Vandenplas,
2013; Vandenplas et al., 2009) may occur.
Based on this information, sound diag-
nosis and considered treatment of GERD
are important goals to provide the best
relief with the least risk of side effects and
to avoid missing another diagnosis. Vari-
ability in clinical practice and particularly
in the use of PPIs is well known. To address
inconsistencies in the clinical practice of
physicians caring for children, the Euro-
pean (ESPGHAN) and North American
(NASPGHAN) Pediatric Gastroenterology
societies published clinical practice guide-
lines for the diagnosis and treatment of GER
in 2009 (Vandenplas et al., 2009). In 2013,
the American Academy of Pediatrics (AAP)
also confirmed and approved the guidelines
(Lightdale Gremse, 2013). Quitadamo
and colleagues (2014) studied the impact
of these documents on the treatment prac-
tices by European primary care family or
pediatric physicians. This group found that
primary care physicians did not implement
the guidelines 82% of the time, and a spe-
cific continuing medical education activity
for primary care physicians resulted in a
significant improvement in PPI prescribing
practices according to the recommenda-
tions. These studies concluded that infants
and children continue to be overdiagnosed
and treated for GERD. Primary care nurse
practitioners have also incorporated these
recommendations into clinical practice
guidelines for their profession (Papachri-
santhou Davis, 2015, 2016).
Experts have made recommendations
regarding the use of PPIs in infants and
children. For normally growing infants with
regurgitation and no warning signs suggest-
ing another underlying diagnosis for the
regurgitation, PPIs should not be consid-
ered unless the regurgitation is ongoing at
age 18 months (Vandenplas et al., 2009). At
that time, a referral for pediatric GI evalua-
tion and endoscopy to evaluate the mucosa
for erosive esophagitis would be in order.
For infants with regurgitation, poor growth,
and no warning signs, the expert recom-
mendation includes workup and treatment
as indicated for undernutrition. If growth
does not normalize, referral for pediatric GI
evaluation, endoscopy, and consideration to
start tube feedings would be the next step.

For children with chronic heartburn and/
or regurgitation, a trial of 2 to 4 weeks of
PPI after counseling about lifestyle changes
related to GER is recommended. If there is
improvement in signs and symptoms, the
PPI could be continued for 8 to 12 weeks
and then discontinued. If the 2- to 4-week
trial does not help or there is relapse after
discontinuing the PPI, referral for pediatric
GI evaluation and endoscopy is in order.
The pediatric gastroenterologist may also
recommend other tests based on the out-
come of the evaluation and endoscopy
(Lightdale Gremse, 2013; Vandenplas
et al., 2009).
Prokinetic Agents. Prokinetic agents
have been used in the past. Significant side
effects combined with a lack of outcomes
research over the last quarter century have
resulted in these medications falling from
favor. Domperidone and cisapride are not
available in the American market and are
not recommended in the global market.
Expert opinion has recommended that
there is insufficient evidence to justify
routine prescribing of metoclopramide,
bethanechol, erythromycin, or baclofen for
infants and children with GERD (Lightdale
Gremse, 2013; Vandenplas et al., 2009).
Surgical Treatment. Surgical treat-
ment is reserved for patients who have
severe GERD unresponsive to medical ther-
apy, recurrent pneumonia, recurrent peptic
strictures, large fixed hiatal hernia, recur-
rent distal esophageal bleeding, or intrac-
table malnutrition. Gastrostomy or jejunos-
tomy tube placement may be indicated to
provide access for nutritional supplements
when infants or children cannot eat enough
orally to thrive. GT placement does not
cause GER or make GER worse afterward
(Heuschkel et al., 2015). Jejunostomy tube
placement may be helpful for patients with
symptomatic delayed gastric emptying or
recurrent pneumonia from aspiration of
stomach contents. Fundoplication, a surgi-
cal procedure to reinforce the LES by wrap-
ping part of the gastric fundus around the
gastroesophageal junction, decreases GER
by increasing baseline LES pressure, increas-
ing the length of esophagus that is located
in the abdomen, decreasing the number
of TLESRs, and reducing a hiatal hernia if
present. There are retrospective studies but
no prospective studies known to this author
on indications for surgery or outcomes after
fundoplication in children. Fundoplication
can be performed with an open, laparo-
scopic, or robot-assisted technique (Ham-
braeus, Arnbjornsson, Anderberg, 2013).
The laparoscopic technique is currently
the most commonly performed in the
United States and has benefits of shorter
hospitalization, less postoperative pain,
smaller surgical scars, and faster recovery
compared to the open technique. A failure
rate of up to 22% has been reported with
fundoplication and often results in the
resumption of long-term use of PPIs (Van-
denplas et al., 2009). Partial fundoplication
may result in less dysphagia than a com-
plete wrap (Weber, 1999). Gastroesophageal
separation is generally reserved for patients
with life-threatening aspiration and pul-
monary compromise or failed fundoplica-
tion, usually in patients who are neurologi-
cally impaired. The procedure eliminates
all GER, allows for primarily gastrostomy
tube feeding postoperatively, is technically
demanding, and carries significant morbid-
ity (Lall et al., 2006).
Eosinophilic Esophagitis
Eosinophilic esophagitis (EoE) presents
with esophageal dysfunction resulting pri-
marily from severe predominantly eosino-
philic inflammation. This field of study is
relatively new and has been noted globally.

For example, a study by van Rhijn and col-
leagues in the Netherlands noted that the
incidence of new diagnoses in childhood
of eosinophilic esophagitis increased from
0.01/10,000 in 1996 to 1.31/10,000 in 2010.
The group acknowledged that increased
awareness and knowledge of this entity
since 2000 created bias in the results (van
Rhijn, Smout, Bredenoord, 2013). It is
a chronic immune/antigen-mediated dis-
ease with a male preponderance of 70% to
80% in both adults and children. Allergy is
present in about 40% of pediatric patients.
Patients present with variable, often vague,
signs and symptoms based on age. Infants
and toddlers often present with feeding
refusals, vomiting, regurgitation, and failure
to thrive. Children present with vomiting,
abdominal pain, or midline chest pain. Ado-
lescents present with dysphagia and midline
chest pain, and may have food impactions,
especially with coarser textures of food.
Visible signs of allergy, such as eczema or
wheezing, may also help steer the clinician
toward this diagnosis. Referral to a pediatric
gastroenterologist for esophagoscopy will
be necessary to make the diagnosis.
Endoscopic Examination for Eosino-
philic Esophagitis. At endoscopy, the
esophageal mucosa may or may not appear
visually normal. The current definition
requires eosinophil counts 15/high power
field (HPF) with basal cell hyperplasia in at
least one epithelial esophageal biopsy and/
or microscopic evidence of eosinophilic
inflammation (Liacouras et al., 2011). Biop-
sies from patients with GERD and peptic
esophagitis usually have eosinophil counts
of 3 to 5/HPF. Patients with counts of 5 to
15/HFP present a treatment dilemma.
Treatment Options for Eosinophilic
Esophagitis. Whilesignificantadvancesin
characterization of eosinophilic inflamma-
tion have occurred in the last two decades,
the body of research to guide clinical man-
agement has lagged. The European Society
of Pediatric Gastroenterology, Hepatology,
and Nutrition (ESPGHAN) and American
College of Gastroenterology (ACG) have
published management guidelines to pro-
vide expert opinions regarding treatment.
Their conclusions are incorporated in the
management described here (Dellon et al.,
2013; Papadoulou et al., 2014).
Dietary treatment is appealing because
it does not involve medication and has been
shown to be effective (Dellon et al., 2013;
Papadoulou et al., 2014). An allergist who
performs food allergy testing and a dieti-
tian skilled in creating palatable allergen-
free diets tailored to a child’s specific allergy
profile are critical members of the treatment
team. (See Chapter 6.) When food allergies
are diagnosed, expert opinion suggests
strict avoidance of those foods (Papadou-
lou et al., 2014). If no specific food allergies
are found by skin prick test, then a six-food
elimination diet (no cow’s milk, soy, wheat,
eggs, peanuts/tree nuts, fish/shellfish) or
introduction of an elemental diet result
in symptomatic relief and less esophageal
inflammation. When numerous food aller-
gies are detected, the easiest balanced diet
may be a proprietary elemental formula.
The main drawback to dietary treatment
is dietary compliance. The specific allergen
and six-food elimination diets may be time
consuming to learn to prepare. In addition,
elemental diets are much less palatable than
food or other liquids.
Pharmacologic treatment alone may be
more convenient than dietary changes. Off-
label treatment with oral budesonide slurry
or orally swallowed fluticasone alone or in
combination with any of the three dietary
recommendations result in improvement
in eosinophil counts, strictures, need for
dilation procedures, and dysphagia (Chan

et al., 2016; Dhaliwal et al., 2014; Oliveira,
Zamakhshary, Marcon, Kim, 2008). Med-
ication often results in noticeable regres-
sion of signs and symptoms within a week.
Patients with intermediate eosinophilic
esophagitis have been treated with topical
steroids and shown improvement with-
out use of PPI (Oliveira et al., 2008). The
main drawback to swallowed topical ste-
roids is intermittent Candida esophagitis,
which is amenable to oral antifungal treat-
ment. Another group of patients may have
PPI-responsive esophageal eosinophilia
(PPI-REE), which is EoE that responds
to PPIs without need for topical steroids
at all. This process is not well understood at
present (Dellon et al., 2013; Papadoulou
et al., 2014).
In summary, EoE is a chronic process,
and relapse of symptoms and inflamma-
tion is common. The current long-term
treatment strategy involves the most palat-
able diet and/or least amount of long-term
medication required to manage symptoms
and esophageal inflammation. Gradual
reintroduction of single foods is key to re-
expanding the diet while pinpointing foods
that trigger recurrence of symptoms. Peri-
odic esophagoscopy is the best way to moni-
tor extent of mucosal inflammation. Esoph-
ageal dilation may be necessary initially for
a symptomatic stricture in conjunction with
weight loss, but not after inducing initial
remission. Rarely, gastrostomy tube place-
ment may be necessary to provide an ele-
mental diet to patients who are highly aller-
gic. Fundoplication is not indicated (Dellon
et al., 2013; Papadoulou et al., 2014) unless
there may be unusual complications.
Eosinophilic Gastroenteritis (EGE)
EGE refers to a chronic relapsing inflamma-
tory disorder characterized by eosinophilic
infiltration of the stomach, duodenum, and
less commonly the small intestines, and the
colon. The prevalence is 22 to 28/100,000
people with a slight male predominance
(Spergel et al., 2011). EGE rarely occurs
in children. It may occur in conjunction
with EoE. The peak age of onset is the third
decade of life. The pathogenesis is not well
understood. Clinical and epidemiologic
features suggest an allergic component.
Children, in particular, with EGE may have
an elevated serum IgE level. For allergic
EGE patients without conventional IgE-
mediated food allergies, an immunological
study has suggested that food exposure may
activate IL-5 expressing food allergen spe-
cific T-helper 2 (Th2) cells leading to gut
eosinophilia (Prussin, Lee, Foster, 2009).
Once eosinophils are recruited to the gut,
they persist by releasing eosinophil-active
cytokines and granulocyte macrophage-
colony stimulating factor (Desreumaux et
al., 1996).
About half of EGE patients have a his-
tory of allergic disease, including asthma,
eczema, specific food sensitivities, or rhini-
tis. The clinical manifestations (Table 5–8)
are related to the location, extent, and
affected layers of the gut (Klein, Hargrove,
Sleisenger, Jeffries, 1970). Patients with
intramural involvement of the stomach or
intestines may also present with mucosal
symptoms. Food protein-induced entero-
colitis syndrome (FPIES) occurs in infancy
as sudden, profuse vomiting and diarrhea
often progressing to dehydration about 1 to
6 hours after eating. Initially, an offending
substance may not be obvious. Although
it is not an IgE-mediated food allergy, the
symptoms are often related to ingestion of
cow’s milk protein, soy, or rice cereal. Once
a food is a trigger, each subsequent inges-
tion results in the same symptoms. A diag-
nosis of EGE is suggested by the presence
of the signs and symptoms in Table 5–8,
peripheral eosinophilia with an absolute

eosinophil count of greater than 500 in the
peripheral blood smear, or history of food
allergies or sensitivities.
Diagnosis of Eosinophilic Gastroenteri-
tis. The diagnosis is confirmed by finding
eosinophilic infiltration in biopsies of the
GI tract or in ascitic fluid, no other affected
organs, and no other causes for eosino-
philia. Imaging by magnetic resonance
imaging (MRI), computed tomography
(CT), or barium contrast may note muco-
sal irregularities or narrowing, but these
findings are not specific or sensitive for the
diagnosis of EGE. Other possible abnormal
laboratory findings include abnormal fecal
fat excretion, abnormal d-xylose testing,
elevated prothrombin time, hypoalbumin-
emia, and iron deficiency in patients with
malabsorption and diarrhea. Serum IgE
is elevated, and erythrocyte sedimenta-
tion rate may be normal or elevated. Other
causes of eosinophilia to rule out before
making a diagnosis of EGE include drug-
induced eosinophilia, hyper-eosinophilic
syndrome, intestinal parasites, Langerhans
cell histiocytosis, malignancy, polyarteritis
nodosa, and Crohn’s disease (Prussin
Gonsalves, 2014).
Treatment Modalities for Eosinophilic
Gastroenteritis. Two treatment modali-
ties have been successful to improve signs
and symptoms and decrease eosinophilia.
The first is dietary management. A pediatric
dietitian with training in creating allergen-
free diets is particularly helpful to improve
compliance by training the family to read
labels on food packages and make palatable
substitutions in the patient’s diet. If specific
food allergies or sensitivities can be identi-
fied, they should be removed from the diet
first. If no specific foods are identified, a
six-food elimination diet may be attempted
for 6 weeks to look for improvement. The
six foods that are usually eliminated are
soy, wheat, egg, milk, peanut/tree nuts, and
fish/shellfish. An elemental diet may also be
prescribed. Infants less than age 7 months
may accept the taste of an elemental diet
Table 5–8. Clinical Manifestations of Eosinophilic Gastroenteropathy
Mucosal Layer
Gi Organ
Involvement Muscular Layer
Subserosal
Layer
Abdominal pain
Nausea/vomiting
Early satiety
Weight loss
Stomach Outlet obstruction
Perforation
Peritoneal fluid
(ascites)
Malabsorption
Fecal protein
losses
Failure to thrive
Small intestine Obstruction
Perforation
Peritoneal Fluid
(ascites)
Diarrhea
Vomiting
Colon (FPIES) Very rare obstruction
Very rare perforation
Note. FPIES = food protein-induced enterocolitis syndrome; GI = gastrointestinal.

more willingly than older children (Men-
nella, Griffin, Beauchamp, 2004). Nonal-
lergenic flavor packets or extracts may be
helpful in disguising the taste. The second
treatment option is glucocorticoids. Glu-
cocorticoids should be considered when
dietary compliance cannot be achieved or
when dietary interventions are not adequate
to improve signs and symptoms. Daily
oral prednisone for 2 weeks followed by a
rapid taper over 2 weeks usually results in
a remarkable improvement in signs and
symptoms. To maintain improvement, the
smallest dose of steroids that will manage
the symptoms is indicated. This treatment
can be accomplished with low-dose pred-
nisone or off-label use of oral budesonide
(Siewert, Lammert, Koppitz, Schmidt,
Matern, 2006).
Infections
Symptomatic esophageal infections are
uncommon in healthy children with nor-
mal immune systems. Affected children
typically complain of heartburn, chest pain,
dysphagia, or pain with swallowing but may
also present with less specific feeding prob-
lems, abdominal pain, or infantile fussiness.
Herpes simplex virus (HSV) and Candida
are the most common infections and may be
accompanied by lesions visible on the lips or
in the mouth. HSV, Candida, cytomegalo-
virus, and even rarer esophageal infections
may occur in immunocompromised chil-
dren with HIV, hematological malignancies
treated with cytotoxic medications, cellular
immune deficiencies, or general debilita-
tion. Damage to the esophageal mucosa by
radiation, caustic ingestion, or impacted
oral medication also increases the risk for
infection. The gold standard for diagnosis
is endoscopy with biopsies and cultures
with serology testing as indicated. Treat-
ment is based on the immunocompetence
of the patient and extent of the infection
(Mohr, 2017).
Structural Abnormalities
(Esophagus)
Tracheoesophageal Fistula and Esoph-
ageal Atresia. Abnormal development
of a separate and fully patent trachea and
esophagus occurs in about 1 in every 3,500
births (Nelson, Green, Olive, 2015). The
embryologic relationships and clinical
implications that result in such anomalies
are discussed in Chapters 2 and 4. About
87% of tracheoesophageal fistula (TEF)
with esophageal atresia (EA) present with a
proximal EA and a distal TEF (Figure 5–4).
This condition is readily recognized soon
after birth when an affected infant is unable
to swallow secretions or oral feedings. NG
tube passage results in coiling in the proxi-
mal esophageal segment. Chest and abdom-
inal x-rays show large amounts of air in the
stomach. Isolated esophageal atresia occurs
in about 8% of cases, and abdominal x-rays
show a gasless stomach. An H-type fistula
occurs in 4% of cases and can be difficult
to recognize until the child becomes seri-
ously ill with recurrent pneumonia during
the first 2 years of life.
TEF and EA occur in the midline of
the body and often present in association
with other congenital anomalies. There-
fore, a careful search for associated prob-
lems is necessary. For example, TEF and
EA are part of the constellation of anomalies
found in CHARGE (coloboma, heart defect,
atresia choanae, retardation of growth and
development, genital abnormality, and ear
abnormality) and VACTERL (vertebral
defects, anal atresia, cardiac defects, tra-
cheoesophageal fistula, renal anomalies,
and limb abnormalities) syndromes. (See

Chapter 12.) Both of these syndromes
include multiple body systems, including
other associated GI abnormalities as sum-
marized in Table 5–9, congenital heart
disease, vascular rings, and genitourinary,
musculoskeletal, and craniofacial anoma-
lies (Koivusalo, Parakinen, Rintala et al.,
2013). Until the 1940s, TEF and EA were
uniformly fatal birth defects. Modern sur-
gical techniques have resulted in marked
improvements of survival rates to greater
than 90% (Koivusalo et al., 2013; Sistonen
et al., 2014).
In addition to highly trained pediatric
surgeons, the skills of many other pediat-
ric specialists and allied health care profes-
sionals are necessary to care for infants born
with midline anomalies. Preoperative radio-
graphic imaging should be performed in
the institution at which the surgical repair
will take place. Neonatal intensivists, with
a dedicated neonatal intensive care unit,
are also necessary for optimal outcomes
during the pre- and postoperative periods
and when complications are likely to arise.
Medical centers treating these patients must
be proficient in primary and secondary
surgical repairs, esophageal replacement,
treatment of surgical complications, and
treatment of esophageal inflammatory dis-
orders and tracheomalacia. Management
of the whole child often requires services to
treat the associated cardiac, GI, nutritional,
nasopharyngeal, orthopedic, urological, or
neurological conditions.
Habilitation to functional swallowing
and feeding can be challenging for some
infants and children. Reestablishing con-
tinuity between the oropharynx and stom-
ach is vital for managing secretions and oral
feeding in infants born with EA. Enteral
feedings may be started after surgery as
soon as the digestive system has recovered
from anesthesia and the respiratory status is
stable. As many as 94% of patients are eating
orally at 2-year follow-up after surgery and
Figure 5–4. A. Distal tracheoesophageal fistulae are most commonly associated with proxi-
mal esophageal atresia. B. Esophageal atresia without tracheal connection. C. Of this group of
anomalies, tracheoesophageal fistula alone, known as an H-type fistula, is the least common.

fewer than 10% remain dependent on GT
feedings (Koivusalo et al., 2013). Patients
may tolerate NG tube feedings and tran-
sition to oral feedings before hospital dis-
charge. For patients who do not progress
as rapidly, GT placement may be necessary
to reach full enteral feedings. Predictors
of poor oral intake include long-gap esopha-
geal atresia, CHARGE syndrome, and neu-
rological abnormalities (Koivusalo et al.,
2013).
Long-Gap Esophageal Atresia. Long-
gap EA, which occurs in about 25% of
patients with EA, is a major predictor of
post-repair complications (Khan et al.,
2009; Koivusalo et al., 2013; McKinnon
Kosloske, 1990; van der Zee et al., 2017).
All patients with long-gap EA require a GT
for feeding. Patients requiring internal or
external traction to elongate the esopha-
geal pouches may have delayed primary
repair for as long as 2 to 10 months after
birth (Khan et al., 2009). Delay in primary
repair results in delays in development
of coordinated suck–swallow oral feed-
ing skills compared to normal infants. In
infants with long-gap EA, learning to drink
with a covered sippy cup and self-feeding of
finger foods correlated negatively with age
at primary repair. They did catch up with
peers’ eating skills over time (Khan et al.,
2009). Oral feeding into a blind proximal
esophageal pouch is technically difficult,
unpleasant for the baby, and poses a risk
for tracheal aspiration. Expert opinion no
longer recommends routine creation of a
cervical esophagostomy to handle secre-
tions as it may complicate the eventual pri-
mary repair (van der Zee et al., 2017). One
reported solution involves use of a Replogle
suction device to allow sham oral feedings
as tolerated in the newborn period while
the esophagus is undergoing traction before
primary repair.
TEF/EA: Esophageal and Gastric Dys-
motility Complications. Esophageal and
gastric dysmotility are known to be abnor-
mal after repair of EA and may contribute to
Table 5–9. Reported Gastrointestinal Anomalies Associated with
Tracheoesophageal Fistula and Esophageal Atresia
Gastrointestinal Organ Anomalies
Esophagus Heterotopic gastric mucosa (inlet patch)
Stomach Dumping syndrome
Duodenum/liver/pancreas Hypertrophic pyloric stenosis
Duodenal atresia or stenosis
Extrahepatic portal vein occlusion
Heterotopic pancreas
Small intestine Ileal duplication
Intestinal malrotation with or without volvulus
Meckel’s diverticulum
Omphalocele
Colon/anus Anorectal malformations (high and low)
Source: Adapted from Koivusalo, Pakarinen, Rintala, 2013.

symptoms of dysphagia. Esophageal motil-
ity has been studied with high-resolution
esophageal manometry (HREM) in 40
children who underwent surgical repair of
congenital esophageal atresia. All children
had abnormal esophageal motility, char-
acterized as pressurization (15%), aperi-
stalsis (38%), or distal contractions (47%).
Dysphagia was a complaint in 83% of the
patients and was noted with all three motil-
ity patterns. None of the motility patterns
was predictive of the presence or severity
of dysphagia (Lemoine et al., 2013). Chil-
dren with aperistalsis in the distal esopha-
gus were more likely to have symptoms of
GER (Kawahara et al., 2007; Lemoine et al.,
2013). HREM combined with impedance
studies has allowed for pressure flow anal-
ysis to study UES and LES relaxation and
bolus movement mechanics (Rommel,
Rayyan, Scheerens, Omari, 2017). More
studies are needed to determine if HREM
will be helpful in directing feeding therapy
to improve dysphagia or identifying which
patients may be more likely to develop com-
plications, such as chronic esophagitis or
Barrett’s esophagus.
TEF/EA: Gastroesophageal Reflux
Complications. Gastroesophageal reflux
is the most common GI tract complica-
tion with a prevalence of 22% to 45% fol-
lowing repair of EA (Krishnan et al., 2014).
For infants and children with isolated EA,
almost all were reported to have GER in an
early retrospective study (Lindahl Rintala,
1995). Manometric studies have demon-
strated that the underlying mechanism for
GER after EA repair is TLESR (Van Wijk,
Knuppe, Omari, de Jong, Beninga, 2013).
Uncontrolled studies suggest that GER is a
major factor for development of recurrent
anastomotic stricture (Banjar Al-Nassar,
2005; Deurloo, Ekkelkamp, Schoorl, Heij,
Aronson, 2002; Koivusalo et al., 2013; Mc-
Kinnon Kosloske, 1990). Most infants
with TEF or EA are treated continuously
with PPIs after undergoing surgical repair.
There are no prospective controlled
studies to the knowledge of this author to
determine how long to treat patients with
acid suppression at any age during child-
hood after EA repair. Shawyer, D’Souze,
Pemberton, and Flageole (2014) concluded
that use of PPI or H2-receptor antagonists
(H2RA) until age 12 months resulted in bet-
ter weight gain and reduced GI or respira-
tory complications. Thus, continuous treat-
ment with PPI until age 1 year to facilitate
better weight gain and to reduce GI and/or
pulmonary symptoms is prudent as long
as the benefits outweigh the risks. At age
1 year, the ongoing extent of GER could
be evaluated by 24-hour pH- or MII-pH
monitoring. If the test is abnormal, contin-
ued PPI treatment is indicated. If the test
is normal, the PPI could be stopped with
regular monitoring for dysphagia or decel-
eration of rate of weight gain. For asymp-
tomatic children, a surveillance endoscopy
may be adequate at age 1 year after stopping
the PPI, one time before age 10 years, and
one time at transition to an adult health care
provider (Krishnan et al., 2016).
ChildrenwithGERthatdoesnotrespond
to adequate doses of acid-suppressing medi-
cations within 4 to 8 weeks may have other
confounding conditions. Esophagoscopy
with biopsies collected above and below the
surgical anastomosis should be collected as
part of the evaluation. A gastric inlet patch
may be easier to identify after previous
treatment for esophageal inflammation.
Differentiation of reflux esophagitis from
EoE in patients with EA can be difficult.
An intermediate level of esophagitis, eosin-
ophilic inflammation that does not meet the
criteria for EoE, is more extensive than that
typical of GER (Oliveira et al., 2008). Two
retrospective studies have reported find-

ings consistent with EoE in biopsies taken
from 17% to 21% of EA patients born since
1999 who continued to have symptoms
after treatment with PPI (Chan et al., 2016;
Dhaliwal et al., 2014). They presented with
chronic GER symptoms, dysphagia, and/
or esophageal strictures. At least half of the
patients with EoE in both groups had a his-
tory of atopy with asthma being the most
common. Food allergies were diagnosed in
30% to 40%. In both studies, symptoms of
dysphagia and GER significantly improved,
and the need to dilate strictures decreased
or ended after treatment of intermediate
esophagitis and EoE with elimination diet,
swallowed topical steroids, or a combina-
tion of the two. Two smaller studies have
reported similar findings of success in treat-
ing recalcitrant GER, dysphagia, or recur-
rent anastomotic strictures when EoE was
found on esophageal biopsies after treat-
ment for peptic esophagitis (Batres, Lia-
couras, Schnaufer, Mascarenhas, 2002;
Oliveira et al., 2008).
The incidence of anastomotic stricture
formation after primary repair of EA is 18%
to 60% (Castilloux, Noble, Faure, 2010;
Kovesi Rubin, 2004; Serhal et al., 2010).
The most frequent presenting signs are dys-
phagia and feeding difficulties. Infants and
children often endure repeated esophageal
dilations for months to as long as 4 years
until the strictures either stabilize enough to
allow advance to full oral feeding or require
surgical treatment to enlarge the diameter
of the esophagus (Wanaguru et al., 2016).
Esophagoscopy with biopsy can be useful to
classify the inflammatory process surround-
ing the stricture. If a confounding process,
such as EoE, food allergies, refractory
reflux esophagitis, or Barrett’s esophagus
is found, medical therapy can be adjusted
(Holschneider, Dubbers, Engelskirchen,
Trompelt, Holschneider, 2007; Huynh-
Trudeau, Maynard, Terzic, Soucy, Bouin,
2015; Ijsselstijn, van Beelen, Wijnen,
2013; Svoboda et al., 2018; Ure et al., 1999).
Figure 5–5 demonstrates the interrelation-
ships of repaired EA with peptic and eosin-
ophilic esophageal inflammation.
If GER is unresponsive to medical man-
agement or if there is poor growth, recur-
rent anastomotic stricture, or recurrent
pneumonia or cyanosis, surgical interven-
tion should be entertained. Patients with
long-gap EA are particularly likely to have
more problems with GER and anastomotic
strictures after primary repair (Wanaguru
et al., 2016). Surgical treatment for GERD
includes partial or complete fundoplication
with or without pyloroplasty or pyloromy-
otomy depending on the type of primary
repair. Pyloric drainage procedures may
result in dumping syndrome and should be
reserved for patients with known delayed
gastric emptying (Holschneider et al., 2007;
Levin, Diamond, Langer, 2011). A suc-
cessful fundoplication may result in even
slower transit of fluids and food through
the esophagus and increased risk for lower
respiratory symptoms. The most common
complication for patients after surgery was
a 16.1% recurrence of GER symptoms, more
than 2.5 times the recurrence rate in a com-
parison group without EA undergoing fun-
doplication. Other complications include
dysphagia and narrowing at the site of
the fundoplication (Holschneider et al.,
2007). There are no reported controlled tri-
als for surgical management of EA patients
with GER.
Innovations in the repair of TEF and
EA, successes in treating complications that
arise after esophageal reconstruction, and
advancesinnutritionalsupporthavecreateda
new population of survivors. The affected
children, young adults, and their supportive
families who have benefited from advanced
care, live with complex and chronic health
conditions. They require regular follow-up

care from a variety of subspecialists and
allied health professionals.
TEF/EA: Health-Related Quality of
Life Associations. Holscher and col-
leagues (2017) reported 154 EA patients
treated over a 50-year period and noted
that 51% of parents felt that during their
child’s initial hospitalization they had
received insufficient information about
what to expect regarding feeding difficul-
ties or potential complications from surgery.
Analysis of focus group discussions with EA
patients to categorize health-related quality
of life (HRQoL) statements and experiences
demonstrated that eating and drinking
issues were most frequently reported. The
feeding problems gleaned from the tran-
scriptions of the discussions include food
issues, impact of choking, nutritional intake
Figure 5–5. Interrelationships of repaired EA with peptic and
esophageal inflammation. (EoE = eosinophilic esophagitis;
GERD = gastroesophageal reflux disorder.) The diagram relates
inflammatory processes that can develop in the esophagus after
EA repair and contribute to stricture formation. 1 = esophagitis
caused by acid reflux, intermediate esophagitis, proton pump
inhibitor-responsive esophageal eosinophilia, or EoE without
stricture.2 = anastomotic stricture formation with reflux esophagi-
tis. 3 = anastomotic stricture formation with EoE. 4 = anastomotic
stricture formation with reflux esophagitis and EoE. Success-
ful prevention and treatment of anastomotic strictures is more
likely with identification and resolution of as much eosinophilic
esophageal inflammation as possible and, if needed, dilation of
symptomatic strictures.

experiences, school cafeteria experiences,
fluid intake experiences, and children’s par-
ties (Dellenmark-Blom et al., 2016).
Pediatric patients born after the 1990s,
young adults born since the 1980s, and par-
ents have been studied to assess quality of
life after repair of congenital EA. The few
published studies have used a variety of
questionnaires, psychological instruments,
or focus group discussions to learn from
patient and family experiences. For patients
with difficult-to-repair EA, one study noted
that the HRQoL of children was comparable
to normal controls, but the Gastrointes-
tinal Quality of Life Index (GIQLI) score
in adults demonstrated significant impair-
ment (p .0001) after any type of EA repair
compared to controls (Dingemann et al.,
2014). Two studies (Dingemann et al., 2014;
Svoboda et al., 2018) have shown that over
time the frequency of complaints of dys-
phagia increase in adulthood compared to
childhood, whereas the frequency of GER
complaints remain the same in adults as in
children with repaired EA.
Health in Adults with CongenitalTEF/
EA. Adults with congenital TEF/EA con-
tinue to face GI health problems through-
out their lives. To address the need for
providing a systematic approach for these
patients born with TEF and EA, the GI
working group of the International Network
on Esophageal Atresia published guide-
lines, based on available scientific informa-
tion and expert opinions (Krishnan et al.,
2016).
Despite a majority of adults reporting
ongoing difficulties with dysphagia, GER
symptoms, and at least one chest infection
and food impaction each year, a survey of
1,100 patients with EA in 25 countries by
Svoboda and colleagues noted that provi-
sion of care decreases over time as children
matureandtransitiontoadulthood.Twenty-
two percent of children with repaired EA
were no longer followed on a regular basis
with a pediatric surgeon by age 5 years. Fifty
percent of adults with EA reported hav-
ing no primary care physician at all (Svo-
boda et al., 2018). Sistonen and colleagues
reported that adults with repaired EA are
at increased risk for chronic esophagitis,
epithelial metaplasia, and development of
Barrett’s esophagus (Sistonen et al., 2010).
A few cases of esophageal adenocarcinoma
have been reported in children and adults
with EA (Cheu et al., 1992). These signifi-
cant chronic health issues in long-term
survivors point to the need for multicenter,
collaborative studies, and creation of a team
approach to manage pediatric and adult
patients with histories of EA. Team follow-
up should at a minimum include at least GI,
pulmonary, surgical, and primary care spe-
cialists. A smooth transition from pediatric
to adult care teams is imperative.
Esophageal Rings and Webs. Esopha-
geal rings and webs are thin, usually delicate,
mucosal structures that partially or com-
pletely occlude the lumen of the esophagus.
Patients generally complain of dysphagia for
solids, especially meat or bread.
Rings usually occur as a single structure
in the distal esophagus, and webs appear in
the anterior cervical esophagus with focal
narrowing in the post-cricoid area. A-rings
occur as smooth muscular rings in the distal
esophagus of children. Once they present,
the symptoms are persistent. B-rings occur
as mucosal rings (LES) and are often asso-
ciated with hiatal hernia. Dysphagia may
be intermittent, and patients may adapt
by chewing longer and extensively or by
choosing to eat foods with softer textures.
If the diameter of the esophagus is 12.5
mm at the LES, then it is called a Schatzki’s

ring. If there is more than one ring, EoE
should be considered.
A web may occur as an isolated struc-
ture or in association with other chronic
disorders, including bullous pemphigoid,
chronic graft versus host disease, epider-
molysis bullosa, pemphigus vulgaris, and
Plummer-Vinson syndrome. During the
evaluation for dysphagia, subtle narrowing
of the esophagus during contrast esopha-
gram or videofluoroscopic swallow study
(VFSS) may suggest the diagnosis of an
esophageal ring or web. Referral to a pedi-
atric gastroenterologist for endoscopy will
likely clarify the underlying cause, and treat-
ment strategies can be planned. If access
to endoscopy is limited, two radiological
methods have been used to try to locate a
web (Chen, Ott, Gelfand, Munitz, 1985;
Smith, Ott, Gelfand, Chen, 1998).
Structural Anomalies
(Stomach and Duodenum)
Pyloric Stenosis. Pyloric stenosis is a nar-
rowing of the muscular sphincter between
the stomach and the duodenum. It occurs
secondary to thickening of the muscles that
make up the pyloric valve. As one of the
most common congenital GI anomalies,
recurrent projectile vomiting, present soon
after birth, is its hallmark. Surgical repair is
warranted and highly successful (Seifarth
Soldes, 2016).
Duodenal Obstruction: Antral or Duo-
denal Webs. Found in 1:6,000 live births
in the United States (Schneider Oldham,
2016), there are three types of duodenal
obstruction: stenosis, web, and atresia.
A recent study noted that 31% of patients
with congenital duodenal obstruction had
duodenal webs (Sarin, Sharma, Sinha,
Deshpande, 2012). Other midline anoma-
lies may also be present. Bilious vomiting
and the characteristic “double-bubble sign”
of air-filled stomach and first portion of
the duodenum seen at birth with duodenal
atresia may have a similar presentation for
a duodenal web later during the first week
after birth. Duodenal web may have a sub-
tler presentation with intermittently bilious
vomiting, abdominal pain, slow weight gain,
and feeding difficulties in infancy or early
childhood. The diagnosis may be missed
initially, especially if another obstructive
bowel anomaly is present. If the web is
only partially obstructive, the duodenum
may be chronically dilated by the time the
web is found. The best diagnostic test is an
abdominal x-ray. If the double-bubble sign
is not present, an upper GI contrast study
should be performed. Depending on the
extent of the web, treatment may be either
endoscopic or an open surgical resection of
the web (Schneider Oldham, 2016).
Dysmotility Disorders
Esophageal Dysmotility (Primary Proxi-
mal Esophagus). The three components
of intact mature esophageal motor function
include an integrated enteric and autonomic
neural system, innate rhythmic smooth
muscle contractions, and propagation of
the peristaltic wave, usually in response to
swallowing, by striated muscle.
Esophageal Motor Disorders Diag-
nosed With High-Resolution Manom-
etry. Abnormal esophageal motility pat-
terns found during esophageal manometry
studies are called esophageal motor dis-
orders. The introduction of high-resolu-
tion manometry (HRM) and improved
capabilities to describe esophageal motor
events resulted in the Chicago Classifica-
tion of esophageal manometric disorders
(Kahrilas, Ghosh, Pandolfino, 2008).
Esophageal motility may be impaired by
four mechanisms: (a) lack of contractions,

(b) absent relaxations (achalasia), (c) exces-
sive contractions (spasms), and (d) unco-
ordinated contractions. Mucosal inflamma-
tion, connective tissue diseases, neurologic
impairment, abnormal muscle function,
and mucosal/muscle replacement, usually
by scarring, are the most common causes
for these motility problems to occur in the
esophagus (Lerner Sood, 2016).
HREM is an important diagnostic tool
in the evaluation of dysphagia to differenti-
ate disorders that may improve after endo-
scopic or surgical procedures from those
that will not. In experienced hands, this out-
patient procedure may be performed safely
in infants and children after intranasal topi-
cal sedation. Findings may contribute to a
treatment path to relieve symptoms. Com-
mon disruptions in motility include crico-
pharyngeal achalasia, esophageal achalasia,
diffuse esophageal spasm, nutcracker esoph-
agus, and nonspecific esophageal motility
disorders, which are discussed in more detail.
Cricopharyngeal and Esophageal
Achalasia. Both forms of achalasia are rare
in children. Delay in diagnosis is common
for both types. Radiologic and manometric
studies are helpful in making the diagnosis
of both types of achalasia. In addition, an
upper GI endoscopy with biopsy is indi-
cated to rule out esophagitis, malignancy,
and associated causes of achalasia in patients
suspected to have esophageal achalasia.
Cricopharyngeal achalasia occurs when
the UES fails to open after initiation of swal-
lowing. The most common presentation is
feeding difficulty, pharyngo-nasal backflow,
and recurrent aspiration. Cricopharyngeal
dysfunction may also be associated with
myoneural junction defects such as myas-
thenia gravis, neuromuscular disorders, and
neural defects (Lerner Sood, 2016).
Esophageal achalasia is characterized
by abnormal esophageal motility and fail-
ure of relaxation of the LES. In one study
of children diagnosed with esophageal
achalasia, over 80% presented with dyspha-
gia and over 50% had recurrent vomiting.
Respiratory symptoms were less common
(Franklin, Petrosyan, Kane, 2014). Dis-
orders associated with esophageal achalasia
include infection with Trypanosoma cruzi
(Chagas disease) and adrenocorticotropic
hormone insensitivity (Allgrove syndrome).
Disorders resulting in either dilation of the
esophagus or narrowing of the distal esoph-
agus and LES produce signs and symptoms
similar to those noted with esophageal
achalasia, such as distal esophageal stric-
ture of any etiology, Nissen fundoplication,
gastric banding, or leiomyoma (Lerner
Sood, 2016).
Treatments for cricopharyngeal and
esophageal achalasia are available. The goal
for both types of achalasia is to provide
symptomatic relief, improve esophageal
transit of food to the stomach, and prevent
chronic dilation of the body of the esopha-
gus. Botulinum toxin injections, balloon
dilation, or surgical myotomy of the upper
esophageal sphincter are the treatments of
choice for cricopharyngeal achalasia when
oral feeding fails (Chun et al., 2013; Huoh
Messner, 2013; Messner, Ho, Malhotra,
Koltai, Barnes, 2011). Several treatment
options are available for esophageal acha-
lasia (Lerner Sood, 2016; Richter, 2013).
Nifedipine, a calcium channel blocker,
taken before meals may offer some symp-
tomatic relief when dysphagia is present.
The mechanism of action is relaxation of
the LES during the subsequent meal. Since
drug tolerance often develops rapidly, nife-
dipine is not a long-term definitive therapy.
It is generally used as a bridge to botuli-
num toxin injections, pneumatic dilatation
of the LES, or surgical Heller myotomy.
Endoscopically injected botulinum toxin
provides improvement by decreasing LES

pressure for a time but must be repeated
at regular intervals to sustain relief. Pneu-
matic dilatation can be performed for older
children, but the dilator is physically quite
large to fit inside the esophagus of an infant
or toddler. It is generally a safe procedure,
but perforation of the distal esophagus may
occur as a complication. Surgical Heller
myotomy for esophageal achalasia is usually
effective for both infants and children. The
most common postsurgical complications
are gastroesophageal reflux and recurrence
of dysphagia. The laparoscopic approach,
when possible, has proven superior to the
open approach with less pain, less scarring,
shorter hospitalization, and more rapid
recovery to normal activities of daily life
(Franklin et al., 2014).
Primary Distal Esophageal Dysmotility
Diffuse Esophageal Spasm. Diffuse
esophageal spasm (DES), a disorder with
strong and prolonged esophageal contrac-
tions with abnormal peristalsis, is rarely a
problem in infants and children. The clini-
cal and manometric findings do not nec-
essarily correlate. A contrast esophagram
may demonstrate a “corkscrew” appearance
typical of localized contractions. In a retro-
spective study by Rosen et al. (2013) of 36
pediatric patients with DES, caregivers or
children younger than age 5 years at diagno-
sis complained of food refusals and vomit-
ing more often than older children. No chil-
dren younger than age 9 years complained
of chest pain. Comorbid disorders were
noted in 33/36 children, including disorders
of the central nervous system (e.g., devel-
opmental delay, Down syndrome, cerebral
palsy, and epilepsy), enteric nervous system
(e.g., neuropathic pseudo-obstruction and
Hirschsprung’s disease), congenital heart
disease, and premature birth. Two children
treated with nifedipine showed improve-
ment in choking and food refusal and suc-
cessful advancement in nutritional support.
The symptoms returned when the nifedipine
was withdrawn a year later (Rosen, Laven-
barg, Cocjin, Hyman, 2013). Adults have
been treated with oral nifedipine. Acid sup-
pression is added when GER is also present
(Burmeister, 2013; Vanuytsel et al., 2013).
Nutcracker Esophagus and Nonspeci
fic Esophageal Dysmotility. Nutcracker
esophagus and nonspecific esophageal
motility disorder, which occur rarely in
pediatric patients, may also be diagnosed
by HREM. Both of these disorders share
the presenting signs and symptoms of epi-
sodic chest pain and dysphagia for solids.
These conditions have distinct manometric
findings that may or may not relate to pain
or dysphagia. Surgery is not indicated to
treat either disorder. There are reports of
treatment with balloon dilation, botulinum
toxin, sildenafil, calcium-channel blockers,
nitrates, and acid suppression for symptoms
related to nutcracker esophagus (Burmeis-
ter, 2013). Nonspecific esophageal motility
disorder has been treated successfully with
acid suppression. Chewing food well, avoid-
ing ingestion of extremely hot or cold foods,
and swallowing a liquid bolus after each
solid food bolus may also increase mealtime
enjoyment for patients with any esophageal
motility disorder.
Stomach and Duodenum Dysmotility.
Dysmotility of the stomach and intestines
may also have untoward effects on swallow-
ing and feeding. Vomiting is common, and
a high index of suspicion should be raised
for this problem when the usual evaluations
and treatments for vomiting are unreveal-
ing or unsuccessful. Evaluation is difficult
and requires a pediatric motility center
with equipment to perform antroduodenal
motility studies. When gastric emptying is a

problem, pyloromyotomy is sometimes rec-
ommended. Fundoplication will not correct
underlying problems with gastric emptying
or other GI motility disorders (Hassall,
2005; Vandenplas et al., 2009).
Miscellaneous Conditions
Compressive Lesions. Masses, cysts, and
major blood vessels intrinsic and extrinsic
to the esophagus can all cause compressive
symptoms and dysphagia. Radiologic eval-
uation consists of a barium esophagram,
CT scan of the chest, and often MRI of the
chest. Treatment will depend on the under-
lying problem.
Constipation. Studies in normal children
with functional constipation indicate that
intake of fluid or fiber exceeding dietary
needs based on age and size does not result
in statistically significant improvement in
constipation (Pijners, Tabbers, Benninga,
Berger, 2009; Tabbers, Boluyt, Berger,
Benninga, 2010, 2011; Tabbers et al., 2014).
Chronic constipation is also a common and
aggravating problem for children with dys-
phagia or feeding disorders. They are par-
ticularly susceptible to this problem because
of decreased overall dietary intake, includ-
ing fluids and fiber. A diagnosis may be
difficult to ascertain because patients with
dysphagia may not neatly fit into the catego-
ries of the Rome III criteria for constipation,
which includes being able to express pain
during defecation or lack of toilet training
to differentiate fecal soiling from defecation
(Hyman et al., 2006; Rasquin et al., 2006).
Impaired ability to walk may contribute
to decreased GI motility and fewer bowel
movements.
Constipation in children with dysphagia
is not well studied. Past studies have esti-
mated that constipation occurred in 26%
to 74% of children with cerebral palsy (Del
Giudice, et al., 1999; Sullivan et al., 2000).
A more recent cross-sectional observational
study (Veugelers et al., 2010) found a preva-
lence of 57% constipation in 152 children in
the Netherlands with severe motor disabili-
ties and moderate to profound intellectual
difficulties. Since patients were not verbal
enough to express presence or absence of
abdominal discomfort or pain, pain was
not assessed as a sign of constipation. One
third of the patients received tube feedings,
but feeding characteristics were otherwise
not reported. Regardless of the presence
or absence of constipation, adequate fluid
and fiber intake based on predicted normal
needs was a challenge for the children with
dysphagia. The prevalence of constipation
was higher in nonambulatory children who
received tube feedings or took medication
with constipation as a side effect. No sta-
tistically significant relationship was found
between fiber or fluid intake and constipa-
tion (Veugelers et al., 2010), a finding in
agreement with studies of functional con-
stipation in normally developing children
(Pijners et al., 2009; Tabbers, et al., 2010,
2011; Tabbers et al., 2014). When other fac-
tors were taken into account, there was no
statistically significant relationship found
between tube feeding and constipation
(Veugelers et al., 2010).
Since constipation is common in chil-
dren with dysphagia, careful attention to
diet is important and should consist of
enough fluid and fiber to meet normal daily
dietary requirements for age and body com-
position. Maintenance stool softener ther-
apy with osmotic action, such as lactulose or
polyethylene glycol 3350, is often very help-
ful. Long-term use of stimulant laxatives
may eventually result in damage to colonic
neurons. In severe cases of stool impaction,
saline enemas to soften the hardened stool
may be helpful. Occasional manual removal

of the stool may also be necessary in the case
of a fecal impaction (Tabbers et al., 2014).
Children with severe neurological im-
pairment often require the assistance of
several caregivers to meet their needs.
To achieve consistent care, collaboration
among medical advisers and in-home care-
givers to identify and resolve barriers to
implementation of any care plan is vital.
A protocol-based regimen clearly stated and
discussed among all caregivers will offer a
better means for successful bowel manage-
ment (Veugelers et al., 2010).
Duodenogastric Reflux. The incidence
of duodenogastric reflux (DGR) was found
to be high in a group of 109 children stud-
ied by 24-hour dual-channel pH probe,
using both gastric and esophageal monitors
(Tovar, Wang, Eizaguirre, 1993). After
clinical testing, 69 (63%) children were con-
sidered to have clinical GER, and 40 (37%)
did not have GER. Of the 69 children with
clinical GER, 40 (57%) were found to have
acid GER, 8 (11%) had alkaline GER, and
15 (22%) acid-alkaline GER. The other six
patients had nonacid GER and were consid-
ered false positives. The refluxate consisted
of gastric enzymes, bile salts, and pancre-
atic enzymes. Alkaline reflux (above pH 7.0)
may have deleterious effects on the mucosa
of the stomach and upper aerodigestive
tract (Tovar et al., 1993). A Belgian study of
six patients with atypical reflux symptoms
reported that all had endoscopically visible
bilious reflux from the duodenum to the
stomach, and 5/6 patients had Helicobacter
pylori-negative gastritis. Five of the six
patients improved within 15 days after treat-
ment with sucralfate, which is widely avail-
able globally, and cisapride, which is not
commercially available in the United States,
with or without concomitant treatment with
omeprazole. One patient required surgical
treatment consisting of duodenal switch
and fundoplication for relief (Hermans,
Sokal, Collard, Romagnoli, Buts, 2003).
It is suggested that DGR be considered in
difficult cases when GERD/ EERD signs are
atypical and endoscopy reveals significant
bilious reflux from the duodenum into the
stomach. The study by Tovar and colleagues
suggests that pH probe testing alone will not
be adequate to detect alkaline reflux unless
gastric pH is also monitored.
Case Studies
Case Study 1
John is a 7-year-old boy with repaired con-
genital tracheoesophageal fistula (TEF)
and no other midline anomalies who pres-
ents with heartburn for 6 months. He has
not had any vomiting, blood in the stools,
rashes, or pneumonia. He points to the
substernal area of the midline of his chest
when asked where the heartburn bothers
him. The heartburn is worse for about 1 or
2 hours after a meal and when he lies down
to sleep after a bedtime snack of milk and a
cookie. Otherwise, he has eaten a balanced
diet of solid and mashed foods appropriate
for age and drinks milk and water every
day. His pediatrician gave him a month-
long therapeutic trial of oral ranitidine with
little improvement in the heartburn. He is
growing normally with weight for height at
the 50%-tile, and he has not lost any weight.
History
Review of history reveals that the surgical
repair of the TEF occurred as a newborn.
The surgeon successfully primarily closed
the fistula and reconnected the proximal

and distal limbs of the esophagus. John
was initially fed by NG tube in the hospi-
tal. With help from the NICU nurses and
speech-language pathologists, he pro-
gressed to total oral breast milk feedings by
bottle at discharge at age 6 weeks. During
his first year of life, John was treated with
a PPI to minimize symptoms of GE reflux
and bronchodilators to minimize wheez-
ing. He advanced to direct breastfeeding
and gained weight with ongoing outpa-
tient feeding therapy. At age 1 year, John
was weaned off the PPI and continued to
grow well. He continued to require inter-
mittent use of bronchodilators for wheezing
with respiratory infections. Acquisition of
developmental milestones for speech and
gross and fine motor movements occurred
at age-expected times. Although his voice
was occasionally hoarse, he acquired speech
and language skills normally. He learned to
eat pureed foods, transitioned to solid foods
at expected ages, and was eating a normal
diet without coughing. At age 1 year, a bron-
choscopy to investigate the hoarse voice was
reported normal and ruled out a laryngeal
cleft or obstruction of the bronchus involved
in the original TEF.
Physical Examination and
Diagnostic Testing
Physical examination at age 7 years revealed
a well-developed, well-nourished boy in
no acute distress. His chest had a midline
vertical, well-healed surgical scar. The
remainder of the physical examination was
normal. A barium esophagram was nor-
mal. VFSS demonstrated no oropharyn-
geal dysphagia or aspiration. A chest x-ray
was normal. There was no occult blood in
the stool. An esophagogastroduodenos-
copy (EGD) demonstrated a midesopha-
geal circumferential scar, corresponding
to the site of the previous surgical repair of
the fistula, with minimal narrowing of the
lumen of the esophagus. Distal to the scar,
the entire circumference of the lining of
the esophagus was very reddened without
ulcerations. Proximal to the scar, the lining
of the esophagus was visually normal. The
mucosa of the stomach and duodenum were
both visually normal. Esophageal biopsies
were taken above and below the scar. The
biopsies of the esophagus demonstrated
extensive eosinophilic inflammation of the
distal esophagus and moderate inflamma-
tion proximal to the scar.
Management Decisions
John was diagnosed with peptic esopha-
gitis and treated with a PPI. The family
and child were counseled about not eat-
ing for at least 2 hours before bedtime and
not eating immediately before strenuous
activity. A recommendation to elevate the
head of the bed for sleep was offered. One
month later, he returned with his parents
and reported that his heartburn was much
less, and his appetite had even improved.
He slept through the night and felt that
he slept more soundly with the head of his
bed elevated.
Comment
Johnisoneof87%ofchildrenbornwithTEF
to have Type 3. EA occurs in the proximal
esophagus, and the TEF connects the bron-
chus to the distal esophageal limb. Surgical
repair of the fistula closes the communica-
tion with the esophagus but leaves an area of
weakness in the wall of the bronchus where
the fistula originally formed. At this loca-
tion, the wall may collapse enough to cause
wheezing or other lower airway breathing
difficulties until the child grows big enough

that the area of weakness no longer func-
tionally compromises the diameter of the
bronchus. The surgical re-anastomosis of
the proximal and distal limbs of the esopha-
gus establishes communication between the
oropharynx and stomach to allow swallow-
ing of oral secretions and food. However,
the neural connections to allow coordinated
propulsion of food toward the stomach are
present only from the oropharynx through
the proximal esophageal limb. That is, the
muscles in the distal esophageal limb and
lower esophageal sphincter do not commu-
nicate with the upper esophageal sphincter
and the propulsive waves of muscle contrac-
tions in the proximal esophagus. Thus, the
distal esophageal limb depends on gravity
to move food, liquids, and medications
into the stomach. Random relaxations of
the lower esophageal sphincter allow food
to reflux into the distal esophagus.
It is important to follow-up patients
with repaired TEF on a regular basis and
assess them for signs of stricture, heartburn,
and swallowing difficulties. If a stricture is
found, gentle esophageal dilation can be
a useful tool to widen the narrowed area.
Children with symptomatic GE reflux will
likely require long-term treatment with
acid-suppressing medications and endo-
scopic examination of the mucosa periodi-
cally. If acid suppression with medication
is not adequate, fundoplication should be
considered.
Case Study 2
Presentation and History
Kaitlyn is a 16-year-old who presented to
the emergency room after eating a roast
beef sandwich that “got stuck” after swal-
lowing. When asked, she stated that this
was not the first time food got stuck after
swallowing, and in the past she had always
been able to get it to pass into her stomach
by drinking water. She admitted that she
ate rapidly and did not usually chew her
food very much. Over the 2 years prior
to presentation, she could remember food
getting stuck five times. The usual culprits
were meats or bread. Liquids and mashed
or semisolid foods never caused any trouble
with swallowing. She had never required
a Heimlich maneuver for respiratory dis-
tress. Kaitlyn sometimes experienced heart-
burn after eating, but she could not iden-
tify any particular foods that precipitated
the heartburn. She had not been treated
by her primary care provider for these
symptoms but a few times she had taken
an over-the-counter PPI for heartburn
after passing food that got stuck. The
medication did not provide much relief.
Over time the heartburn seemed to lessen
spontaneously until the next time food got
stuck.
Physical Examination and Testing
On physical examination in the emergency
room, she was a well-developed, well-
nourished, mildly anxious young woman.
She drooled saliva into an emesis basin
at times. There was no sign of respiratory
distress, and the remainder of the examina-
tion was normal. She went to the endoscopy
suite, was sedated with general anesthesia
and intubated to protect the airway, and a
bolus of a sandwich was removed endo-
scopically from her distal esophagus. The
gastric and duodenal mucosae were nor-
mal. The esophageal mucosa was reddened
and edematous throughout. Biopsies were
obtained. Greater than 40 eosinophils per
high-powered field were noted throughout
the proximal and distal esophagus. A bar-
ium esophagram after clearance of the food
impaction was normal, and there were no

findings to suggest that she had achalasia,
hiatal hernia, or a stricture. A chest x-ray
was normal.
Treatment and Follow-Up
She was treated with a daily PPI and was
instructed to eat more slowly, take appro-
priately sized bites, and chew and swallow
before taking the next bite. She returned
for follow-up in 2 months and her symp-
toms had resolved. A second endoscopy
was recommended to assess the healing of
the esophagus since she had such extensive
inflammation on the first one. At the second
endoscopy, she continued to have greater
than 40 eosinophils per high-powered field
in the proximal esophagus but the distal
esophagus was only minimally inflamed.
No hiatal hernia was noted. A 24-hour
dual-channel pH probe study did not dem-
onstrate acid reflux.
New Diagnosis
Kaitlyn was diagnosed with eosinophilic
esophagitis (EoE). She was treated with
dietary restriction of milk products, con-
tinuation of the PPI, and added fluticasone
without the spacer given orally twice per
day. She continued to feel well. Two months
later, the PPI was discontinued, and she felt
well on a milk-free diet and twice daily
fluticasone.
Comment
For patients who have a food impaction,
follow-up is essential. Since no particular
reason for the impaction was identified, an
accurate diagnosis was needed to decide if
or when to stop PPI therapy. After resolu-
tion of the inflammation from the initial
impaction, another examination was war-
ranted. By making a more accurate diagno-
sis of EoE, treatment could be modified to
manage the inflammation more effectively.
Case Study 3
Brandon is an otherwise normally healthy
8-week-old infant born at term who had
one occasion at home of apnea, circumoral
cyanosis, and relaxation of muscle tone
during sleep. His family called 911, started
CPR, and Brandon was pink and crying vig-
orously by the time the paramedics arrived.
The episode had occurred within 30 min-
utes after a breastfeeding. According to his
parents, he did not vomit and did not have a
seizure during the episode. He typically lies
in his bed on his back and does not roll over.
His examination in the emergency room
was normal. There was no other pertinent
medical, family, or social history.
Comment
In 2016, the American Academy of Pedi-
atrics (AAP) published clinical practice
guidelines to help clinicians clarify situ-
ations when young infants have a single
event with at least one of the following four
symptoms: brief apnea, pallor, decrease in
muscle tone, or altered responsiveness (Tie-
der et al., 2016). First, they recommended
that the name “apparent life-threatening
event” (ALTE) be changed to “brief resolved
unexplained event” (BRUE). They also rec-
ommended that the event characteristics
be addressed by name rather than simply
called ALTE. Second, episodes of BRUE
were divided into low- and high-risk cat-
egories. Low-risk events were defined by

(a) age 60 days, (b) gestational age at birth
32 weeks and corrected gestational age 45
weeks, (c) no CPR by a trained professional
required, (d) event lasted 1 minute, (e) no
prior events, (f) normal physical examina-
tion, and (g) no positive pertinent history.
All other events were defined as high risk.
Next, the AAP made recommendations
about the evaluation and treatment of chil-
dren with low-risk BRUE. First, clinicians
should educate parents about BRUE and
collaborate with caregivers to make a plan
for evaluation, disposition, and follow-up.
They should also offer resources to caregiv-
ers to learn CPR. Clinicians may do pertussis
testing, 12-lead electrocardiogram (ECG),
and/or serial observation to include moni-
toring with pulse oximetry in the outpatient
department for a short time. The guidelines
do not recommend blood or urine testing,
radiologic testing, endoscopy, EEG, anti-
epileptic or acid-suppressing medication,
or hospitalization for infants with low-risk
BRUE. Patients whose event is deemed high
risk should be evaluated and treated for the
identified cause of the event.
Case Study 4
Zoe is a 7-year-old otherwise healthy girl
who presented with acute onset of difficulty
swallowing all solid foods after choking on
French fries with catsup 1 week ago. She was
able to cough out the food and since that
episode will drink only liquids, primarily
milk. She did not have any cyanosis during
the choking episode and did not require a
Heimlich maneuver. She has lost 3 pounds
in 1 week and has developed some constipa-
tion since drinking more milk than usual.
She has had no changes in urination. She
has not had any breathing difficulty since
choking. Her current diet consists of whole
milk, occasional fruit juice, water, diluted
smooth-pureed fruit or vegetable smooth-
ies, and popsicles. She gags when offered
any solid food and says she is going to
choke. She has not missed any school and
does not otherwise appear to be ill as long as
she drinks only liquids. Review of systems
was otherwise unremarkable.
Physical Examination and Testing
On physical examination, she was a well-
developed, well-nourished anxious girl
in no acute distress. The remainder of the
examination was normal. Barium esopha-
gram and VFSS were normal. An upper GI
endoscopy was also normal.
Diagnosis and Follow-Up
A diagnosis of choking phobia was made,
and she was referred to a psychologist for
treatment.
At follow-up in 6 weeks she was eating
normally again, had regained the weight she
lost, and constipation had resolved without
medications.
Comment
Sudden onset of fear of swallowing in a
child who was eating normally can be
very anxiety-provoking for patients, fami-
lies, and medical professionals, especially
when there is weight loss. Choking pho-
bia is often related to a specific episode of
choking on food or pills. When the patient
presents for treatment soon after the onset
of the symptoms, the history may be easier
to elicit before memories begin to fade.
Timely testing to make a diagnosis and rule
out pathology in the digestive system will
facilitate prompt referral to a psychologist
for treatment.

Case Study 5
Christopher, an 8-month-old boy, was
referred for feeding difficulties and fail-
ure to progress from liquids to purees. He
was the family’s first child, born at term
after an uneventful pregnancy and deliv-
ery. The neonatal screen was normal. He
was initially breastfed successfully until
age 4 months when his mother returned to
work. He made a transition to formula with-
out difficulty but gagged on smooth purees
when they were introduced at age 6 months.
By age 8 months he was taking only minis-
cule tastes of thin puree mixed with formula
from a spoon and formula by bottle. He was
not rolling over or sitting independently.
His weight for height was at the 25%ile and
he was steadily gaining weight. His head cir-
cumference was steadily increasing propor-
tionally with his height and weight.
His physical examination was normal for
age,andtherewasnoobviousdysmorphism.
Follow-Up
Over the next 3 months, the rate of weight
gain slowed, and the formula was calorically
fortified. Christopher contracted viral pneu-
monia over the winter and lost weight. As
he recovered, he was unable to eat enough
to regain the weight. His weight for height
dropped to 3%ile. He was evaluated by a
speech-language pathologist and found to
have inefficient oral skills and some tongue
weakness. He was crawling but not pulling
to stand or walking along furniture. A neo-
natal screen to identify inborn errors of
metabolism was repeated and was normal.
Tube feedings were discussed. His parents
felt that they could not manage NG tube
feedings. A gastrostomy tube was placed.
He was enrolled in physical, occupational,
and speech/feeding therapy and made some
progress with gross and fine motor skills.
He thrived while relying on tube feedings
to gain weight. Oral feeding remained time-
consuming with small volumes, and trials of
an H2-antagonist and later a PPI made no
difference with eating. By age 15 months he
was walking. Over time, he progressed to all
oral feeding except during illnesses. Chris-
topher continued to receive occupational,
physical, and speech/feeding therapy. At
one appointment, he bumped his head and
his mother said, “He’s always clumsy.”
Status at Age 3 Years
At age 3 years, he started an early childhood
program and continued to make develop-
mental progress. When his mother com-
plained that he had frequent headaches, an
MRI of his head was completed. The results
were negative. A trial of cyproheptadine
was started. He ate better and the headaches
mostly resolved. At a follow-up appoint-
ment to discuss timing to remove the feed-
ing tube, he sat quietly and played with his
hands, repeatedly dislocating the joints in
his fingers and cracking his knuckles. At
that point, his mother noted that he “cracks
his knuckles all the time.”
Additional Diagnostic
Workup With Genetics
Upon further questioning, his mother
was also “double jointed,” did tricks for
her friends by contorting her hands and
ankles as a youth, and had several shoul-
der dislocations over time. She and Chris-
topher were referred to a genetics clinic
for further evaluation. They and several
other family members were diagnosed with

Ehlers-Danlos syndrome (EDS). He contin-
ued to take cyproheptadine. The gastros-
tomy tube was successfully removed before
he started kindergarten.
Comment
Sometimes, the hardest patients to treat
have vague symptoms that do not seem
to fit neatly into a single diagnosis. Zarate
and colleagues (2010) have described unex-
plained GI symptoms and joint hypermobil-
ity. The differential diagnosis of dysphagia
is long, and many of the genetic causes of
dysphagia are rare. While clinicians do their
best to treat the issues at hand even when
no unifying diagnosis may ever be found,
ongoing vigilance to look for hints at a uni-
fying diagnosis may be fruitful. Physical
examination was not helpful in this case as
babies and young children normally have
more mobile joints than adults. The absence
of physical dysmorphism also did not signal
a genetic cause for dysphagia. The best indi-
cator to entertain a diagnosis of EDS was
a positive maternal history when Christo-
pher’s mother was asked directed questions
about hypermobility. Hakim and Grahame
(2003) devised five questions to screen
patient families for hypermobility, includ-
ing the following:
1. Can you now (or could you ever)
place your hands flat on the floor
without bending your knees?
2. Can you now (or could you ever) bend
your thumb to touch your forearm?
3. As a child, did you amuse your friends
by contorting your body into strange
shapes or could you do the splits?
4. As a child, or teenager, did your knee
cap or shoulder dislocate on more
than one occasion?
5. Do you consider yourself “double-
jointed”?
Answering yes to two or more questions
suggests joint hypermobility with a sensi-
tivity of 85% and specificity of 90% (Hakim
Grahame, 2003).
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237
6Pediatric Nutrition
Mary Beth Feuling and Praveen S. Goday
Summary
All children need and are entitled to ade-
quate nutrition. Optimal nutrition supports
growth, appropriate health, and neurode-
velopment. Nutritional supervision is a
critical part of support for healthy growth,
and this supervision becomes more vital in
children with special health care needs and
feeding problems.
Accurate assessment and reassessment
of nutrition and feeding skills are keys to
the nutritional care of children with special
health care needs. Nutrition is the backbone
of the management of children with feeding
difficulties. Ultimately, nutrition interven-
tions must tie in with the overall care of all
children and address the needs of children
and their families. Thus, a team approach
may be required in the care of children with
swallowing and feeding problems.
Introduction
The goals of this chapter are to describe
typical childhood nutrition to the feeding
specialist. Understanding typical childhood
nutrition will serve as a foundation for the
discussion of the assessment of nutrition
status and management of nutrition and
growthconcerns.Second,themajornutrition
interventions that can be pursued in children
with feeding difficulties are outlined.
Normal Nutrition
Premature Infants
Specialized nutritional intervention plays a
major role in making survival possible in
extremely premature infants. The optimal
growth rate for premature infants is the rate
at which they would have grown in utero.
Children born before 30 weeks’ gestation
(or 1200 g birth weight) benefit from early
parenteral nutrition (nutrition delivered
intravenously) started in the first few hours
of life (Trivedi Sinn, 2013). For the prema-
ture infant, this parenteral nutrition replaces
the nutrition that the child was receiving
via the umbilical artery from the mother.
This parenteral nutrition is continued until
enteral feedings (nutrition delivered into the
gastrointestinal tract) can be started based
on medical stability and gradually advanced
to goal feedings. Children born before 32 to
34 weeks’ gestation cannot be expected to

feed totally orally. Once they reach 32 to 34
weeks’ gestation and are medically stable,
oral feeding either in the form of breast- or
bottle-feeds should be attempted (Chap-
ter 7). Enteral nutrition should be contin-
ued until oral feedings reach goal volumes
with adequate efficiency.
Breast milk has significant benefits for
premature infants. Presently, most neonatal
intensive care units (NICUs) in the United
States are also using banked human milk
to feed premature infants who do not have
access to their own mother’s milk (Com-
mittee On Nutrition, Section On Breast-
feeding, Committee On Fetus Newborn,
2017). However, breast milk by itself does
not provide complete nutrition to pre-
mature infants (born at 34 weeks gesta-
tional age or 2 kg birth weight). These
infants require breast milk fortifiers that
add energy, protein, vitamins, and miner-
als to the human milk (Moro et al., 2015).
When human milk is unavailable, formulas
that are designed for premature infants are
preferred over standard infant formulas.
Formulas designed for premature infants
provide added energy, protein, calcium, and
phosphorus to the infant. Premature infants
should be discharged on postdischarge pre-
mature formulas (which are different from
the formulas designed for hospitalized pre-
mature infants) that should be continued
up to 12 months corrected age (chronologic
age corrected for prematurity).
Full-Term Infants
It is recommended that infants be breast-
fed exclusively for the first year (Section on
Breastfeeding, 2012). In addition, both the
American Academy of Pediatrics (AAP) and
the World Health Organization (WHO) rec-
ommend that solid foods be delayed until
about6monthsofage(complementaryfeed-
ing). Introduction of solids before 4 months
of age is associated with excessive weight
gain and adiposity, both in infancy and
early childhood (Weng, Redsell, Swift, Yang,
Glazebrook, 2012). When breast milk is
not available, standard cow’s-milk-based
infant formula may be used. Other infant
formulas are also available (Table 6–1). For-
tified infant cereal is an optimal first food
for infants because it is an excellent source
of iron. During weaning from an all-liquid
diet, small amounts of new foods should be
introduced. Single-ingredient foods should
be introduced one at a time every 4 or 5 days
before introducing another new food. By 7
to 8 months of age, infants should be eat-
ing from all the food groups. This should
lead to optimal dietary diversity as children
get older. Over time, the texture of foods is
gradually advanced as oral sensorimotor
and swallowing abilities become more fully
developed (Chapter 7). As infants grow, it is
best to let them self-regulate their intake of
food to prevent future obesity.
Toddlers and Older Children
Cow’s milk and water are the ideal bever-
ages for children. Cow’s milk should not be
started before 1 year of age (Agostoni et al.,
2008). Optimal daily cow’s milk intake is
16 oz for 2 to 3 year olds; 20 oz for chil-
dren ages 4 to 8 years, and 24 oz between
the ages of 9 and 18 years (U.S. Department
of Health and Human Services, 2015). Some
children with feeding disorders may require
other formulas that can be given orally or
via enteral tubes (Table 6–2). Juice should be
limited to no more than 4 oz per day; fruit
is preferred to even 100% fruit juice (Hey-
man Abrams, 2017). Toddlers should be
provided with appropriate portions of foods
from all food groups and should maintain
regular eating schedules and be allowed to

239
Table 6–1. Breast Milk and Infant Formulas
Preterm infants
Breast milk
• Gold standard but fortification required to achieve needs
• Should be used if available
Human milk fortifier
• Should be given to infants 1800 g and/or 34 weeks gestational age
• Can only be used as an additive to breast milk
• Used to increase the protein, vitamin, and mineral content of breast milk
• For hospital use only
Premature formula
• Used in the absence of breast milk
• For hospital use only; children should be discharged on transition formula
Premature discharge formula (transition formula)
• Used in premature infants 36 weeks gestational age and given until 12 months
corrected gestational age
• Standard concentration provides 22 kcal/oz; may be concentrated up to 30 kcal/oz
• Provides more calcium, phosphorus, and protein than standard infant formula
Term infants
Breast milk
• Gold standard; should be used if available and able to achieve nutrition needs
• Used with vitamin/mineral supplementation to meet nutrition goals/needs
• May be concentrated up to 30 kcal/oz using term infant formula
Cow’s-milk-based formula
• Contain cow’s milk protein and lactose
• Standard concentration provides 19 or 20 kcal/oz; may be concentrated up to 30 kcal/oz
Soy-based formula
• Contain soy protein; does not contain lactose
• Indications: galactosemia; transient deficiency of lactase; infants with specific allergies
to cow’s milk but not allergic to soy protein; strict vegetarians or hereditary lactase
deficiency; should not be used in premature infants
Low-lactose formula
• Less lactose than standard formulas
• Indications: Infants with minor feeding intolerances
Added-rice formula
• Contain cow’s milk protein with added rice that causes it to thicken in the stomach
• Indications: gastroesophageal reflux
Extensive protein hydrolysate formula
• Cow’s milk protein-based formulas that have been “broken” down and are considered
hypoallergenic
• Indications: milk protein intolerance, impaired intestinal function, liver disease
continues

240
Elemental formula
• Contain free amino acids and are considered most hypoallergenic
• Indications: milk protein intolerance, food allergy, eosinophilic esophagitis, severe
malabsorption
Specialty infant formulas
• Additional specialty formulas to treat specific diseases including impaired renal
function, metabolic disorders, lymphatic dysfunction, and carbohydrate intolerance are
available
Table 6–2. Formulas for Children Over 12 Months of Age
Pediatric formulas
Milk-based formula
• Standard formulas that provide 1 kcal/ml
• Contain cow’s milk protein and are lactose free
Soy-based formula
• Contain soy protein and provide 1 kcal/ml
• Indications: milk allergy, vegetarian diets
Reduced-calorie formula
• Typically provide 0.6 kcal/ml
• Indications: low energy needs
High-calorie formula
• Provide 1.2 kcal/ml, 1.5 kcal/ml or 2.0 kcal/ml
• Decrease volume of formula that needs to be administered which may (a) decreased
time required for feeding and (b) increase time between feedings to improve appetite
Hydrolyzed protein formula
• Milk protein-based formulas composed of hydrolyzed protein that provide 1.0 kcal/ml,
1.5 kcal/ml
• Indications: malabsorption, liver or pancreatic dysfunction
Elemental formula
• Contain free amino acids, are hypoallergenic, provide 0.8 to 1.0 kcal/ml (may be
concentrated up to 1.5 kcal/ml)
• Indications: food allergy, eosinophilic esophagitis, severe malabsorption
Whole-food formula/homemade blended food
• Formulas made from a wide variety of protein sources—prepared with whole foods
providing a range of calorie concentrations
• Caution: must be evaluated to determine if it is providing complete balanced nutrition
• Indications: delayed gastric emptying, history of retching, caregiver desire to provide
table food by tube
Other specialty pediatric formulas
• Additional specialty formulas to treat specific diseases including impaired renal
function, metabolic disorders, lymphatic dysfunction, and carbohydrate intolerance
are available

6. Pediatric Nutrition 241
Adult formulas
• Used for children 13 years of age
• Available in all categories that are available with pediatric formula
• Range of calorie concentrations are available; primarily ready-to-feed formulas that
do not require mixing
feed themselves and, hence, control the
amounts they eat. Toddlers typically need
two to three healthy snacks a day to meet
nutritional requirements in addition to
meals and a nutritious beverage, typically
cow’s milk. Picky eating and food jags (eat-
ing only one favored food or a very small
group of foods for every meal) are common
among toddlers (Chapter 13). Both concerns
are best managed by continual exposure to
the foods that have been refused previously;
these repeated exposures usually lead to
acceptance. Choking on food is primarily
a concern in children up to 4 years of age
(or developmental levels up to this age). The
foods that are typically implicated as high
risk for choking are round, hard, and do not
dissolve in saliva such as hot dogs, grapes,
and nuts. These foods should not be offered.
The following are general guidelines for
feeding children (Council on School Health,
Committee on Nutrition, 2015):
n Select a mix of foods from the five food
groups: vegetables, fruits, grains (pref-
erably whole grains), low-fat milk and
dairy, and quality protein sources (e.g.,
lean meats, fish, nut butters, eggs).
n Offer a broad variety of food
experiences.
n Limit highly processed foods.
n Use the minimum amount of added
sugar necessary to facilitate the
enjoyment and consumption of
nutrient-dense foods (U.S. Department
of Health and Human Services, 2015).
n Offer portion sizes that are appropriate
for the child’s age (U.S. Department of
Health and Human Services, 2015).
Adolescents
Specific nutrition concerns during ado-
lescence are the increased consumption
of energy through the consumption of
energy-dense but nutrient-poor foods. This
practice places adolescents at risk of obesity
while not meeting requirements for essen-
tial micronutrients, such as calcium, iron,
and zinc. In addition, adolescents are likely
to indulge in alternative diets such as veg-
anism. Adolescents should be encouraged
to consume a variety of nutrient-dense
foods. Adolescents indulging in alterna-
tive diets should be counseled on how to
obtain optimal nutrition while still follow-
ing their specific dietary preferences. They
may also need micronutrient supplemen-
tation to meet their needs while following
these diets. Nutrition needs from preterm
infants through adolescence are described
in Table 6–3.
Dietary Diversity
Dietary diversity is defined as the number
of different foods or food groups consumed
over a particular period of time (Ruel,
2003). Lack of dietary diversity is a problem
among the poor in the developing world

because their diets are based primarily on
starchy, nutrient-poor foods. Their diets
include little to no high-protein foods, fresh
fruits, or vegetables. Recent increases in the
consumption of highly processed foods in
the developed world have led to concerns
about the lack of dietary diversity. This lack
of diversity is particularly true of children
with feeding problems.
Normal dietary diversity has not been
well defined. It is known that food groups
play a role in helping to understand dietary
diversity and is the starting point for
determining if there is adequate nutrition.
MyPlate is one example of a distribution
of food groups that is considered a goal
for dietary diversity (Figure 6–1). There is
a wide range of what would be considered
adequate depending on the cultural back-
ground of the child. Thus, cultural appro-
priateness should be a major factor in deter-
mining adequate dietary diversity. There are
many reasons for limited dietary diversity
that include, but are not limited to, inability
to consume the food (food allergy, lack of
feeding skill, etc.); refusal to eat the food;
food faddism/elimination of specific foods.
Limited dietary diversity can contribute to
problems with growth or may lead to micro-
nutrient deficiency.
Table 6–3. Estimated Nutrient and Energy Recommendations From Premature Infants
Through Adolescence
Nutrient
Premature
37weeks
gestational
age
Infant
0–12 months
Toddler
1–3 years
Child
4–10 years
Adolescent
11–18 years
Energy 110–135
kcal/kg
55 kcal/kg 55 kcal/kg 40–47 kcal/
kg
25–30 kcal/
kg
Protein 3.0–4.0 g/kg 1.2–1.52 g/kg 1.05 g/kg 0.95 g/kg 0.85–0.95 g/
day
Vitamin D 400 IU/day 400 IU/day 600 IU 600 IU 600 IU
Calcium 200–260
mg/day
200–260 mg/
day
700 mg/day 1000–1300
mg/day
1300 mg/day
Iron 11 mg/day 7 mg/day 8–10 mg/day 8–10 mg/day
Zinc 2–3 mg/day 2–3 mg/day 3 mg/day 5–8 mg/day 8–11 mg/day
Vitamin C 40–50 mg/
day
40–50 mg/
day
15 mg/day 25–45 mg/
day
45–75 mg/
day
Fluid As per the
Holliday-
Segar
equation*
*The Holliday-Segar equation is used to calculate fluid requirements based on body weight as follows:
10 kg 100 ml/kg
10–20 kg 1000 ml + 50 ml/kg for each kg above 10 kg
20 (−80) kg 1500 ml + 20 ml/kg for each kg above 20 kg

Vitamin and Mineral
Requirements
All infants and children should receive at
least 400 IU (International Units) of vita-
min D per day beginning soon after birth.
Hence, all breastfed infants should receive
400 IU of supplemental vitamin D daily
(Wagner, Greer, American Academy of
Pediatrics Section on Breastfeeding and
Committee on Nutrition, 2008). This rec-
ommendation also holds for formula-fed
infants, until the infant is consuming at least
32 oz of formula a day. Exclusively breastfed
infants should also receive 1 mg/kg per day
of oral iron beginning at 4 months of age
until appropriate iron-containing comple-
mentary foods (including iron-fortified
cereals) are introduced (Baker, Greer,
Committee on Nutrition, American Acad-
emy of Pediatrics, 2010). Iron supplemen-
tation is not needed for infants receiving
standard infant formulas. Most children
do not require vitamin and mineral supple-
mentation beyond infancy as long as stan-
dard dietary guidelines are being followed.
However, children with feeding problems
and malnutrition may benefit from vitamin
and mineral supplementation.
Infants and Children
With Special Needs
Infants with developmental delay or mul-
tiple handicaps usually have nutrient needs
similar to those of typically developing
infants. These infants can meet their nutri-
tional needs through oral feeding with
standard infant formulas. Some infants
Figure 6–1. Example of distribution of food groups. MyPlate
illustrates the five food groups that are the building blocks for
a healthy diet using a familiar image. (From U.S. Department
of Agriculture. ChooseMyPlate.gov Website. Washington, DC.
MyPlate. https://origin.www.cnpp.usda.gov/MyPlate.htm)

with oral sensorimotor dysfunction exhibit
problems in the neonatal period, whereas
others may have functional suck/swallow/
breathe sequencing for the first few months
with problems becoming evident when
transition feeding occurs and solid foods
are introduced.
Infants need alternative modes for nutri-
tion when suck/swallow/ breathe sequenc-
ing is not functional or when dysphagia
or other conditions preclude oral feeding.
Nonnutritive sucking should be encouraged
for infants who are unable to eat (Chapter 7).
As infants grow, oral sensorimotor incoor-
dination of suck and swallow may become
exaggerated because of pulmonary/airway
problems, behavioral factors, or other devel-
opmental delays. Optimal methods of feed-
ing children with poor or delayed ability
to suck and swallow should be determined
by appropriate evaluation. When total oral
feeding is not possible, optimal methods for
providing hydration and nutrition must be
addressed.
Older children with special needs may
require increased energy for a variety of
reasons. Some children may have reduced
energy needs. Individual variation in caloric
requirements is so great that general guide-
lines for assessing energy requirements are
not possible, and close monitoring of weight
gain in each individual is essential.
Nutrition Screening
A basic nutrition screen may start the
process of identifying children at risk for
nutritional problems. Nutrition screening
is a process to identify an individual who
may be malnourished or at risk for mal-
nutrition to determine if a comprehensive
nutrition assessment is indicated (ASPEN,
2015). When screening identifies children
with severe problems, referrals for further
investigation and treatment should follow.
Given some basic knowledge of nutrition
and growth, public health nurses, as well
as teachers, therapists, and social workers,
should be able to perform a basic nutri-
tion screen. Weight, height, and head cir-
cumference measures can be plotted on the
appropriate WHO growth standards for
children from birth to 2 years of age (CDC,
2013). For children older than 2 years of
age, the 2000 Centers for Disease Control
and Prevention (CDC) growth charts must
be used (CDC, 2013). These growth charts
should be used for charting weight, length,
head circumference, weight-for-length (for
children under 2 years), and body mass
index for age (BMI) (for children 2 years
of age), and repeated at regular intervals.
Weight-for-length and BMI are important
indicators of proportionality and can be
used to assess appropriateness of weight
for an individual child. In addition to paper
charts that can be downloaded, electronic
versions may be accessed, such as through
an electronic medical record or WHO
anthro (a downloadable program) (WHO,
2011) or an internet-based program, Pedi-
tools (Chou, 2017), or as an electronic app
(STAT GrowthCharts, compatible with iPod
Touch, iPhone, iPad) (StatCoder, 2017).
One of the advantages of electronic ver-
sions is the accessibility of z-score calcula-
tions. While growth charts are available for
several specific populations (e.g., prema-
ture infants and Down syndrome) (Fenton
Kim, 2013; Zemel et al., 2015), standard
growth charts should probably be used to
monitor children who may be at nutritional
risk. Nutrition screening programs can pro-
vide anticipatory guidance and educational
materials to families with a goal for preven-
tion of nutritional problems.

Nutrition Assessment
Nutrition assessment is a comprehensive
approach to defining the nutrition state
that uses a combination of the following:
medical, nutrition, and medication histo-
ries; physical examination; anthropomet-
ric measurements; and laboratory data
(ASPEN, 2015).
Medical History
A comprehensive review of present prob-
lems, medical and surgical histories, and
review of systems is important to help
determine etiologies of poor nutritional
status. When specific nutritional disorders
or inheritable metabolic disease are found,
referral is made to the appropriate special-
ist. For example, children with phenylke-
tonuria (PKU), maple syrup urine disease,
cystic fibrosis, and sickle cell anemia are all
at risk for nutrition deficiencies. Further-
more, dietitians should routinely screen for
swallowing problems as part of the standard
nutrition assessment. This practice may aid
in decreasing dysphagia-related complica-
tions (Brody et al., 2000).
Nutrition History
This part of the nutrition assessment helps
to determine a usual dietary pattern or
nutrient intake. It is usually requested that a
diet history or log is maintained by the care-
givers for 3 days: 2 weekdays and 1 weekend
day. They are asked to record all food and
liquid consumed including portion sizes,
time and duration of meals, and bowel
movements. These data are used along with
pertinent history information related to
feeding that might influence dietary intake.
It is also critical to ask questions regarding
caregiver food/beverage beliefs and restric-
tions both within the household as well as
specifically related to the child. If specific
foods are avoided, reason for the avoidance
must be understood.
A diet history is an essential part of the
assessment. Information about physical
activity is also recorded so that both caloric
intake and energy expenditure can be calcu-
lated. The clues from the history that may
suggest feeding difficulties are in Table 6–4.
Children with feeding difficulties may
have appropriate weight gain and growth,
malnutrition, or excessive weight gain lead-
ing to overnutrition. Irrespective of overall
nutrition status, children with feeding dif-
ficulties may have inappropriate distribu-
tion of macronutrients of carbohydrate,
fat, and protein that leads to nutrition risk.
In addition to macronutrients, children
with limited dietary diversity either due
to their feeding problem or due to medical
diagnosis, such as food allergy, are at risk
for inadequate micronutrient intake. The
micronutrients in question are dictated by
the child’s specific situation and must be
assessed and addressed. Many times, there
will not be signs of deficiency on physical
exam and early intervention can prevent
severe deficiency.
Behavioral and Feeding Skills
The influence of development of feeding/
swallowing and behavior on nutritional
status cannot be underestimated. This is so
important that these skills often are evalu-
ated by a feeding specialist (e.g., speech-
language pathologist and/or occupational
therapist) or a child psychologist. Historical
feeding information that is obtained by any

of the members of the care team that relate
to skill, behavior, and nutrition will provide
clues that may suggest and/or support the
feeding difficulties (see Table 6–4).
The nutrition status of the patient should
also be assessed using a nutrition-focused
physical examination (NFPE) (Green Cor-
kins, 2015). Evidence of subcutaneous fat
loss, muscle loss, and the presence of edema
should be sought. Additionally, physical
signs of micronutrient deficiency should be
looked for. Early identification of changes
in nutritional status with use of NFPE
improves outcomes for pediatric patients
avoiding the negative impact to their growth
and development (Figures 6–2, 6–3, and
6–4). It is essential that NFPE is performed
by a trained professional in combination
with complete nutrition assessment, which
would also include medical record review,
anthropometrics, and diet/nutrition intake.
Anthropometric Measurement
Anthropometric measurement refers to the
measures of body dimensions and relative
fat and muscle composition. Anthropo-
metric measurements are quick, accessible,
and inexpensive, in identifying acute and
Table 6–4. Historical Clues That May Suggest Feeding
Difficulties
Feeding skills*
• Any delay or difference in advancement of textures
• Texture-specific volume or variety issues
• Slow chewing
• Coughing, choking, gagging
• Effortful swallow, compensatory swallowing
• Meals 5–10 minutes or 30 minutes
Behavioral/psychosocial*
• Child cannot tolerate nonpreferred food near him/her
• Consistently refuses to try variety of food
• Significant behavior problems/tantrums during meals
• Grazing throughout the day
• High parent stress around mealtimes
Growth and nutrition
• Altered growth
• Feeding intolerance
• Overreliance on liquid/food groups
• Requirement for enteral tube feeding
*Note. Some of the historical clues may actually be caused by
another domain, for example, prolonged mealtimes may be second-
ary to skill problems or behavioral issues.

chronic nutritional status. No single mea-
sure is sufficient to characterize nutrition
status. Furthermore, it is important to com-
pare the various measurements over time.
As previously mentioned, growth charts are
used to track weight for age, height (length)
for age, weight-for-length for age, BMI, and
head circumference for age.
The growth of infants born before 36
weeks’ gestation should be tracked on the
Fenton growth charts until they reach 50
weeks’ postmenstrual age (Fenton Kim,
2013). Thereafter, the growth of these infants
can be tracked using the WHO growth stan-
dards (CDC, 2013). When standard WHO
growth standards are used, a corrected age
should be used for plotting weight, length,
and head circumference until a child reaches
24 months chronologic age.
Anthropometric measures of growth
have traditionally been reported in com-
parison with population data as percentiles.
With the easier availability of electronic
tools, it is now recommended that z-scores
(or standard deviation scores) be used to
facilitate comparisons of anthropometric
Figure 6–2. Physical exam for identification
of subcutaneous fat loss. Orbital region—sur-
rounding the eye.
Figure 6–3. Physical exam area for identifica-
tion of muscle loss.Clavicle bone region—pec-
toralis major, deltoid, trapezius muscles.

data with population data. Hence, z-scores
are now recommended for the assessment
of nutritional status in children (Mehta
et al., 2013). A z-score represents the num-
ber of standard deviations that a specific
data point is above or below the mean (or
50th percentile). The 50th percentile is
equal to a z-score of 0, and so data points
above the 50th percentile are positive while
data points below the 50th percentile are
negative. The second percentile is roughly
a z-score of −2, while the 98th percentile is
roughly a z-score of +2. The 25th and 75th
percentiles are −0.67 and +0.67, respectively.
Z-scores can be used to describe children
under the first (or over the 99th) percentile
and should be used to describes changes in
anthropometric data over time (e.g., a drop
from the 50th percentile [z-score: 0] to the
25th percentile [z-score: −0.67] is a drop in
0.67 z-scores).
Mid-upper arm circumference (MUAC)
for age z-score has become a recommended
indicator for monitoring nutrition sta-
tus. It is a primary indicator for diagno-
sis and documentation of undernutrition
and should be used in the care of children
(Becker et al., 2015). WHO standards are
recommended for children 6 to 59 months
of age (de Onis, Yip, Mei, 1997). For chil-
dren older than 59 months, standard devia-
tions have recently been reported (Abdel-
Rahman, Bi, Thaete, 2017). MUAC has
been shown to be more sensitive to changes
in fat and muscle mass than BMI in adults
(Powell-Tuck Hennessy, 2003).
In sick premature infants, weight mea-
sures are recommended daily. Length and
head circumference are measured weekly.
Changes in fluid balance occur rapidly in
these premature infants and can greatly
alter body weight, so trends in growth over
time are important. Appropriate growth is
measured by an increase in all body com-
partments. Unfortunately, standards are
not available for triceps skin fold or MUAC
measurements in premature infants or for
infants up to 3 months of age.
Full-term infants should be weighed
to the nearest 0.01 kg with no diaper or a
dry diaper on a table beam or digital infant
scale (Figure 6–5). Older children should be
weighed to the nearest 0.1 kg with little or
no outer clothing and no shoes (Figure 6–6).
Length is measured to the nearest
0.1 cm. Children less than 2 years of age
are measured in supine position. Lengths
should be obtained with the infant in supine
position on a measuring board, with the
Figure 6–4. Physical exam area for identifi-
cation of muscle loss. Patellar region—quad-
ricep muscle.

249
Figure 6–5. Infant in a dry diaper being weighed on a digital infant scale.
Figure 6–6. Child being weighed in a seated position, espe-
cially useful for children who cannot stand on a regular scale.

head held securely against the stationary
headboard, the legs in full extension, and
the slide moved to press firmly against the
bottom of the feet. Children older than
the age of 2 years and who are able to
stand are measured in standing position.
A weight-for-length for age (or BMI for age)
z-score is then obtained. The nutritional
assessment is made on the basis of weight
in relation to current height. This weight-
for-length or BMI for age z-score is more
meaningful than weight for age.
Acute versus chronic malnutrition needs
to be defined to determine cause(s). In acute
malnutrition, a low weight-for-height (or
BMI) is the first noticeable sign, usually
with the z-score being less than −1. This
measurement reflects the relatively short-
term onset of slowed weight gain when
length velocity is maintained in the normal
range. Acute malnutrition may be caused
by a recent onset of illness, by a change in
nutritional needs, or by alterations in nutri-
tional intake. In contrast, chronic malnutri-
tion is characterized by low weight and low
height/length. Here, the height z-score is a
more important consideration than either
the weight or the weight-for-length (or BMI
for age) z-scores. In these cases, slow height
growth due to chronic malnutrition must
be distinguished from slow growth due to
genetic factors, such as short stature or con-
stitutional delay in growth.
Head circumference should be mea-
sured routinely with a flexible metal or non-
stretchable plastic-coated tape. This mea-
surement is especially important during the
first 2 years of life because of the rapid brain
growth during that time. Head circumfer-
ence is usually maintained in children with
mild-to-moderate malnutrition. Only in the
case of chronic severe malnutrition is there
a decline in head circumference z-scores.
When a child presents with microcephaly
or a small head circumference, investigation
is needed because it may be due to other
medical conditions.
Most children grow along defined per-
centiles or z-scores of a growth chart. When
there is a drop in z-scores of 0.7 particu-
larly of weight and weight-for-length (or
BMI for age), nutritional inadequacy of the
diet should be explored. In these children,
if the diet is adequate, other medical con-
ditions that could be responsible for the
decline in z-scores should be investigated.
Anthropometric Measurements in
Children With Physical Disabilities
Weight and height are sometimes difficult to
obtain with children who have physical dis-
abilities (e.g., scoliosis or joint contractures).
When older children are unable to stand,
a wheelchair scale can be used (Samson-
Fang Bell, 2013). Ulnar (forearm) length
can be measured using designated calipers
and this can be used to calculate height and
monitor linear growth (Gauld, Kappers,
Carlin, Robertson, 2004). Equations that
calculate body height from knee height
and tibial length are available in some age
groups (Chumlea, Guo, Steinbaugh, 1994;
Stevenson, 1995).
Specific growth charts are available to
monitor the weight of children with cerebral
palsy. These growth charts are categorized
by the Gross Motor Function Classification
System (GMFCS) and the sex of the patient
(Brooks, Day, Shavelle, Strauss, 2011).
On these charts, weight below the 20th per-
centile (marked as a red zone) is thought
to be associated with increased morbidity
and mortality. In the children most severely
affected with cerebral palsy (CP) (GMCFS
V), these charts can indicate an unhealthy
weight where tube feeding may be ben-
eficial. They also may indicate a low but

healthy weight (above the red zone) where
these children can be maintained with tube
feeding (Table 6–5).
The general use of weight-for-length (or
BMI for age) z-scores in children with CP to
determine nutritional adequacy is fraught
with difficulty. Children with CP have
altered muscle and bone mass that makes
these assessments unreliable (Kuperminc
et al., 2010). Similarly, use of skinfold thick-
ness in children with CP to assess fat stores
is also problematic. These children store fat
centrally (such as in the abdominal cavity),
and skinfold thickness may underestimate
fat stores (Kuperminc et al., 2010).
Laboratory Data
In the past, serum proteins (albumin and
prealbumin) were considered nutritional
markers. They should no longer be consid-
ered a reflection of present nutritional status
or current dietary intake. Those biochemi-
cal measures have been shown to be nor-
mal in malnourished children with anorexia
nervosa, cerebral palsy, and AIDS (Hender-
son, Talusan, Hutton, Yolken, Caballero,
1997; Lark et al., 2005). Concerns about
specific micronutrient deficiencies should
prompt targeted laboratory measurements
that may aid in diagnosis and management.
Malnutrition
Updated criteria are available to diagnose
malnutrition in children. These criteria can
be further divided into criteria that apply
where the child is being seen for the first
time with a single data point and criteria
where previous anthropometric data are
available.
Table 6–5. Factors Affecting Energy Needs in Children
With Cerebral Palsy
• Inadequate or excessive nutrient/energy intake
• Increased energy needs due to increased work to
maintain normal tone and posture
• Altered absorption
• Behavior disturbances
• Decreased appetite
• Feeding/volume intolerance
• Poor dentition
• Altered growth pattern related to genetic condition (e.g.,
Down syndrome)
• Associated medical conditions
• Disorders that affect oral, nasal, or pharyngeal function
• Aerodigestive disease, airway or pulmonary
• Congenital and other heart disease
• Neurologic, developmental, and psychiatric disorders
• Other gastrointestinal disorders

The criteria when the child is being seen
for the first time include weight-for-length
(for children 2 years of age) or BMI-for-age
z-scores, MUAC z-scores, and length/height
z-scores (Table 6–6). Solely using anthro-
pometric measurements (and/or nutrition
intake data) to diagnose malnutrition can
be problematic. Some element of subjective
assessment of the overall child may be nec-
essary. For instance, the consideration that
children with either a weight-for-length (or
BMI for age) or MUAC z-score that is above
−2 (or roughly the third percentile) are mal-
nourished should be approached with some
skepticism. Using these criteria would mis-
label some children who are not malnour-
ished as malnourished. Similarly, defining
height solely by a z-score and not taking
into account parental heights can be prob-
lematic. This criterion is likely to exclude
some children who are malnourished but
have a z-score greater than −3.
When prior anthropometric data are
available, criteria include weight gain veloc-
ity, weight loss in children older than 2 years,
decline in weight for length/BMI for age
z-score, and inadequate nutrient intake of
either energy or protein (Table 6–7). The
percentage of expected weight gain in the
first year of life can be misleading when
children are seen at short intervals. In the
study that led to the creation of the WHO
Table 6–6. Criteria for Identifying and Diagnosing Malnutrition Related to Undernutrition:
Initial Evaluation With a Single Data Point
Measures
Malnutrition
Mild Moderate Severe
Weight-for-height or BMI for age z-score −1 to 1.9 −2 to −2.9 ≤ −3
Length/height-for-age z-score — — ≤ −3
Mid-upper arm circumference z-score −1 to 1.9 −2 to −2.9 ≤ −3
Note. Adapted from Becker et al., 2015; 2014 Pediatric Malnutrition Consensus Statement.
Table 6–7. Criteria for Identifying and Diagnosing Malnutrition Related to Undernutrition:
Follow-up Evaluation With Two or More Available Data Points
Measures
Malnutrition
Mild Moderate Severe
Weight gain velocity (2 years) per
WHO data
75% of
expected
50% of
expected
25% of
expected
Weight loss (2–20 years) of usual
body weight
5% 7.5% 10%
Decline in weight-for-length/ BMI z-score ↓1 z score ↓2 z scores ↓3 z scores
Inadequate nutrient intake (% of
estimated energy/protein needs)
51–75% 26–50% ≤25%
Note. Adapted from Becker et al., 2015; 2014 Pediatric Malnutrition Consensus Statement.

growth standards, small amounts of weight
loss were noted in some typical children
when they were measured 4 weeks apart at
the end of the first year of life (WHO, 2017).
The drop in weight-for-length or BMI-for-
age z-scores seems excessive; a drop in one
z-score (this equates to a drop from the
75th percentile to the 37th percentile) is
necessary in order to be considered mildly
malnourished. Finally, inadequate nutrient
intake requires the presence of a dietitian or
a person who is skilled in estimating energy
and protein intake to assess the presence of
malnutrition. In addition, estimated needs
can vary significantly.
In summary, these are good starting
points, but further assessment and some
subjectivity in the criteria for malnutrition
are required. Apart from considerations
previously described, thought should be
given to parental size and parental growth
patterns.
Standard definitions of malnutrition do
not include overweight and obesity. In chil-
dren, overweight is classified as BMI for age
between 85th and 95th percentile for age
and sex, while obesity is BMI for age ≥95th
percentile for age and sex (Barlow, 2007).
Severe obesity is a BMI ≥120 percent of the
95th percentile values, or a BMI for age ≥35
kg/m2
(whichever is lower) (Barlow, 2007).
This definition of severe obesity is recom-
mended because it is practical, and because
the CDC growth curves are not sufficiently
precise at the 97th and 99th percentiles
(Barlow, 2007).
Interdisciplinary Assessment
When evaluations of all of the previous
areas are completed, the registered dietitian
summarizes the findings with caregivers
and other professionals. Optimal interven-
tions/goals take into account the oral sen-
sorimotor feeding skills, risks for aspiration
or airway problems, health concerns, fam-
ily food habits, and the child’s preferences
where applicable. Often it is critical that the
feeding specialist and the registered dieti-
tian collaborate to ensure interventions will
support all goals that are associated with
feeding progression.
Nutritional Interventions
in Malnutrition
Initial steps to enhance nutrition status
focus on improving oral nutritional intake.
This is usually done in the form of increased
energy provision. Almost all children bene-
fit from increased energy provision through
the form of a calorie-dense beverage. In
infants, this can be done by adding infant
formula powder to breast milk (to increase
the calorie concentration from 20 to 24 or
27 kcal/oz) or by decreasing the amount of
water used to make infant formula (thus
increasing calorie concentration to 24 or
27 kcal/oz). In children older than 1 year
of age, 30 kcal/oz formulas may be recom-
mended or additives can be used to increase
the concentration of whole milk to 30 kcal/
oz. Another strategy is to increase the calo-
rie density of solid foods. This can be done
by adding a variety of fats (e.g., oils, but-
ter) to the solid foods that the child con-
sumes. This strategy is better than provid-
ing energy-dense nutrient poor foods (junk
foods) so that the child does not become
accustomed to junk foods and can resume
consuming regular food, once malnutrition
has been treated.
Some children are unable to consume
the energy they need in order to maintain
optimal nutrition and growth. Tube feeding
should be considered for those children.

Typically, children who require short-term
tube feeding should be fed via nasogastric
(NG) tube. If tube feeding is required for
more than 2 months, gastrostomy tube
(G-tube) feedings are ideal (Braegger et al.,
2010). A variety of formulas are available
for tube feeding. In addition, the use of
whole food formulas or homemade tube
feeding made from table foods may also be
an option.
Some children fail to tolerate G-tube
feeding noted by vomiting and failure to
grow adequately and maintain nutrition sta-
tus. These children may require continuous
tube feeding as opposed to bolus feeding.
If children fail to tolerate continuous tube
feeding, they may be candidates for jejunos-
tomy tube (J-tube) feeding. Children who
fail to tolerate any form of enteral feeding
or have intestinal failure are candidates for
parenteral nutrition (Chapter 5).
A general schematic to determine nutri-
tion interventions in children with malnu-
trition is shown in Figure 6–7.
Nutrition assessment
Inadequate intake Adequate intake
Monitor weight and
growth
Inadequate
weight
gain
Adequate
weight
gain
Further
medical
evaluation
Continue
monitoring
Trial oral nutrition
supplementation
Follow intake and
weight
Intake remains
inadequate
Improved intake with
adequate weight gain
Evaluation for
NG tube/G-tube
placement
Close monitoring
of intake and
weight
G-tube
placement, if
feeding needed
for 8 weeks
NG tube placement, if
feeding needed for 8
weeks (with continued
re-assessment)
Figure 6–7. Enteral nutrition decision tree. Steps in evaluation and management decision-
making when children demonstrate poor growth/undernutrition. (NG tube: nasogastric tube;
G-tube: gastrostomy tube.)

Children Receiving Significant
Nutrition Support Therapy
Whenever possible, children should receive
pleasurable oral stimulation with tastes as
safe and tolerated. Similarly, all children
should be encouraged to take in as much
nutrition and hydration by mouth as sup-
ported by their medical, feeding skills, and
developmental status. When children are
receiving most or all of their nutrition par-
enterally or via J-tube feedings or have sig-
nificant vomiting, oral intake beyond some
degree of oral stimulation with minimal
tastes may not be possible. They should be
assessed regularly for the ability to progress
to oral feeding.
Feeding options related to tube depen-
dence are on a continuum. J-tube depen-
dence should be monitored, and if toler-
ated, the pump rate is gradually increased
with a decrease in time receiving feedings. If
medically appropriate, next is to trial feed-
ings by G-tube via continuous drip. Again,
if tolerated, pump rates are increased to
give interval feedings with breaks. This is
the transition to mimic a more typical feed-
ing schedule, which prepares the child for
oral feeding and proves volume tolerance.
The last and final step is to transition to
bolus feedings and if tolerated to give these
feedings over 20 to 30 minutes, again to
mimic oral feeding durations and intervals
between feedings.
Transition From Enteral
Feeding to Oral Feeding
Cues that help provide guidance to readi-
ness for transition to oral feeding include
changes in tube feeding that demonstrate
volume tolerance. If a child is receiving
continuous drip feedings, this results in
decreased appetite. An hourly rate suggests
volume tolerance with the following:
60 ml/hr = 2 oz tolerated over 1 hour;
1 oz over 30 minutes
Feeding specialists must ask the ques-
tion, “Is the volume tolerated over 30 min-
utes?” To decrease tube dependence and
increase oral intake, there must be a plan to
establish readiness for weaning. This plan
includes review of the patient’s medical/
nutritional stability, volume tolerance, abil-
ity to establish schedule for oral eating, pro-
vide appropriate texture and energy goals
while monitoring, anthropometrics/growth
and fluid and energy intake. If the child
has been assessed to be ready to wean, it is
common to decrease energy intake by tube
by, appropriately, 5% to 25% increments
depending on patients’ skill and ability to
take in oral energy. Discontinuation of tube
feeding should be considered when oral
intake meets 75% of the energy goal, pro-
vides adequate hydration, all medications
are taken orally, oral intake supports appro-
priate growth for 2 months, and there has
been no use of tube feeding during illness.
There is often a need to give supplements
such as a complete multivitamin with iron to
assist with meeting micronutrient needs, as
well as it is common for fluid to be given by
tube for a longer period of time than energy.
Summary
In conclusion, all children with feeding diffi-
culties must have regular nutritional assess-
ments. They often require a team approach

to manage the range of feeding challenges
and provide the necessary interventions. It
is important to maximize the health of these
children by ensuring adequate nutritional
status while maximizing their oral feeding
potential.
Children with special health care needs
will thrive with a variety of feeding and nutri-
tion plans. Their goals and interventions
must be customized to meet the needs of the
child at that moment in time. As the child’s
medical status changes, his or her nutrition
needs also change and must be reconsidered.
Therefore, it is crucial that each member of
the care team keeps the impact of their next
recommendation on the larger picture for
that child’s feeding difficulties, especially the
impact on the child’s nutrition status.
Case Studies
Case Study 1
“Janice,” a 20-week-old female infant, born
at 24 weeks’ gestation, is now at a corrected
age of 4 weeks. Weight is 3.5 kg, and length
is 52 cm. Her weight and length should be
plotted on the growth chart as a 4-week-old
infant (40 weeks normal gestation minus
24 weeks actual gestation = 16 weeks pre-
mature; 20 weeks chronologic age minus 16
weeks premature = 4 weeks corrected age).
On the NCHS growth chart as a 4-week-old
infant, she is at nearly the 25th percentile
for both weight and length, with weight-for-
length at the 25th percentile. She is there-
fore growing quite well and is probably
not malnourished. In contrast, if weight
and length are plotted at her chronologic
age of 20 weeks, she would be below the
5th percentile for both weight and length,
which would lead to an erroneous interpre-
tation that she is growing poorly. Weight
is adjusted up to 24 months, height up to
40 months, and head circumference up to
36 months (Frank, Needlman, Silva, 1993;
Kraus Mahank, 1984).
Comment
This case is an example of a preterm infant
and how the corrected age is used to evalu-
ate nutrition status.
Case Study 2
“Joseph” presented at 6 months of age.
History revealed a term infant with nor-
mal prenatal and postnatal course. He was
breastfed exclusively until 4 months of age
and then transitioned to cow’s milk formula
at that time, when mom returned to work.
He developed watery stools at this transi-
tion, and these stools have not resolved. He
weighs 6.16 kg and is 64.2 cm in length.
His head circumference is 43.3 cm, and
his MUAC is 120 mm (z-score: −2.07).
His Wt/Ht z-score = −1.93; Wt gain veloc-
ity = 75% of norm. He is acutely mildly
malnourished as noted by not meeting the
appropriate weight gain velocity for age
and MUAC z-score between −2 and −3. His
poor growth has not impacted his linear or
head circumference growth at this time.
Given a strong family history of food
allergies, the child was suspected to have
cow’s milk protein hypersensitivity. He was
switched to a protein hydrolysate formula
and this resulted in resolution of diarrhea.
The formula switch also resulted in rapid
catch-up weight gain.
Comment
This case study reveals the critical need to
determine the etiology for the undernutri-
tion, and not simply to boost calories, with-

out appropriate medical assessment. This
child would have been harmed further if he
had continued with foods that he could not
absorb adequately.
Case Study 3
“Paul,” a 2-year-old with CP, gets his nutri-
tional needs met with a combination of oral
feeding with pureed foods supplemented by
formula via G-tube. He requires 1,200 calo-
ries per day. On a specific day he took in
800 calories by mouth from pureed foods.
He thus requires an additional 400 calories
that can be given as a continuous overnight
tube feeding of a 1 kcal/ml formula, given as
40 ml/hr for 10 hours overnight.
Comment
This method ensures a consistent daily
intake despite inevitable variations in a
child’s oral intake. It also has the advantage
of freeing the child from the tube during
the more active daytime. Typical eating
patterns throughout the day can be encour-
aged. Gradually the G-tube feeds should be
shifted to bolus feeds if Paul can tolerate the
increased volume in shorter time periods.
Case Study 4
“Rosa” is a 12- year-old girl with autism who
only accepted three foods: full-fat yogurt
(strawberry-banana flavor), fruit leather,
and Boost Breeze, a juice-like, fat-free nutri-
tion beverage. The strawberry-banana fla-
vored yogurt was removed from the market,
and his mother was unable to purchase it for
him. This child had laboratory manifesta-
tions of essential fatty acid deficiency but no
overt clinical signs or symptoms. Since this
child was seen by an interdisciplinary feed-
ing team, the psychologist was able to get
him to accept other flavors of full-fat yogurt
and alleviate the deficiency.
Comment
This is an example of the impact of a severely
restricted diet due to feeding refusal. In
this case, by no longer having access to
the yogurt and her refusal to eat any other
foods, this resulted in her taking in inad-
equate fat resulting in nutrition risk. Close
medical attention and appropriate interven-
tion helped resolve the problem.
Case Study 5
“Tony” is an 8-year-old who presented to
the hospital with a weight loss of 5% over
the previous 3 months due to increased
seizure activity and ongoing feeding diffi-
culties. His weight was 19.3 kg (z-score =
−2.3), height was 133 cm (z-score = 0.79)
and BMI for age: 10.9 kg/m2
(z-score =
−6.91) and MUAC 168 mm (5%–10%ile).
Parents reported that he had always taken
his nutrition orally but required pureed
foods and drank a 1 kcal/ml beverage. His
consumption of both purees and liquids had
decreased over the prior 3 months. Parents
are spending at least 6 hours each day trying
to feed him. His physical exam supports the
diagnosis of severe malnutrition. Parents
are asking for help and want some sort of
tube that will provide him the nutrition he
needs. After discussion with the parents, the
decision is made to have a G-tube placed
during the hospitalization to provide sup-
plemental nutrition.
Comment
This child has had an increase in energy
needs due to increased seizure activity. The

ongoing seizure activity and associated
medical and developmental concerns are
associated with his inability to increase his
oral intake. These factors have led to weight
loss and malnutrition. Along with the sup-
plemental nutrition he will receive through
his G-tube, he should continue to work with
a feeding skills specialist and a dietitian to
continue to optimize oral intake.
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261
7Clinical Swallowing and
Feeding Assessment
Introduction
The clinical evaluation of infants and chil-
is typically the starting point for clinicians to
begin sorting out multiple factors that may
be underlying the observable characteristics
noted during a feeding session. The World
Health Organization (WHO) description of
an International Classification of Function-
ing, Disability, and Health (ICF) (2001) sets
the stage for an in-depth discussion of clini-
cal feeding evaluation procedures, interpre-
tation, and management decision-making.
The clinical swallowing and feeding evalu-
ation process is based on concepts from the
ICF and expanded in 2007 to add Children
and Youth Version (ICF-CY), Centers for
Disease Control and Prevention (CDC)/
National Center for Health Statistics (2007).
ICF provides a framework to address func-
tioning and disability related to a health
condition within the context of the individ-
ual’s activities and participation in everyday
life. This framework is used for measuring
health and disability at both individual and
population levels (Table 7–1). It focuses on
the impact of conditions and enables com-
parisons across all conditions by using a
common metric. The metric is the impact
on functioning of the individual. In infants
and children, that impact on function-
ing extends to caregivers as well. The ICF
framework goes beyond a purely medical or
biological conceptualization of dysfunction
and takes into account other critical aspects
of disability. This approach allows for the
impactoftheenvironmentandothercontex-
tual factors on functioning to be considered,
analyzed, and recorded (WHO, 2001, 2007).
Screening tools and guidelines that
have been developed in recent years pro-
vide guidance for parents and primary care
providers to determine needs for a clini-
cal feeding evaluation. A primary goal is
early identification that in turn may aid in
prevention of the development of severe
chronic feeding/swallowing problems and
their associated consequences.
The clinical evaluation allows for iden-
tification and delineation of the attributes
that are observable while infants and chil-
dren are drinking and eating to whatever
extent possible for them. Clinical evalu-
ation is usually carried out in person, but
for children with limited access to quality
health care, another option may be tele-
health (Kantarcigil, Sheppard, Gordon,
Friel, Malandraki, 2016). It is important
to remind readers at the outset of this chap-
ter that it is not possible to define pharyn-
geal swallowing function by clinical obser-
vation. Pharyngeal swallow physiology can

only be inferred during a clinical feeding
evaluation. Instrumental swallow exami-
nations are necessary to define pharyngeal
and upper esophageal phases of swallowing
(Chapter 8).
The clinical feeding observation is often
limited for children dependent on tube feed-
ings for most or all of their nutrition and
hydration needs. Nonetheless, these obser-
vations provide a baseline for clinicians to
determine next steps in the development
of management decisions to advance oral
feeding skills safely and efficiently. Under-
lying etiologies should be delineated so that
all interrelating factors can be integrated
for a “whole child” approach to manage-
ment/intervention. All professionals from
multiple disciplines must have extensive
knowledge across these multiple systems.
At the same time, each professional must
make decisions that are within the scope of
practice and ethical guidelines. Clinicians
of all disciplines, especially nonphysicians,
should continually remind themselves that
this area of practice has a high risk for
negative impact on cardiopulmonary or
gastrointestinal status of infants and chil-
dren. Improper diagnosis or mismanage-
ment places these children in jeopardy for
poor nutrition and impaired health status
that may include reduced energy and lower
long-term cognitive, communication, and
Table 7–1. International Classification of Functioning (ICF), Disability and Health— Model
to Describe Constructs and Considerations for Infants and Children With Swallowing and
Feeding Disorders
Constructs Considerations for Pediatric Swallowing and Feeding Disorders
Body functions
and structures
Functions: Sucking, swallowing, biting, chewing; sensorimotor skills;
positioning; cognitive/communication; physiologic stability
Structures: Anatomy, physiology, neurophysiology of oral, pharyngeal,
and upper esophageal structures; also laryngeal and other airway
structures; gastrointestinal tract
Activity and
participation
Activities involved in drinking from bottle or cup, eating age-appropriate
food; use of utensils; body positioning
Need to determine whether adaptations/modifications are needed in
areas of self-help to include positioning alterations, special utensils, limited
textures in diet; modifications in strategies to optimize levels of activity
Participation includes family mealtimes, social situations, and
educational settings; need to determine strategies to promote
inclusiveness in all environments with adaptations as needed—
examples include children on tube feedings and those who need
additional assistance in getting food and liquid to the mouth
Environmental
and personal
factors
Consideration of caregiver/family understanding of the child’s feeding/
swallowing disorder; access to appropriate food, liquids, utensils,
seating/positioning equipment
Determine caregivers’ willingness and ability: to prepare food and liquid
in modified forms, to use special utensils and seating systems, apply
special strategies to help the child eat and drink safely
Consider cultural and society judgments of the family with a child
having swallowing and/or feeding disorder

7. Clinical Swallowing and Feeding Assessment 263
sensorimotor outcomes. Well-thought-out
and coordinated planning can enhance the
lives of these children and their caregivers.
It takes a team to evaluate and manage
these children at high risk for multiple com-
plications across a wide range of medical,
surgical, neurodevelopmental, cognitive/
communication, and behavioral factors.
Thus, no clinician should evaluate and
treat these children with swallowing and
feeding problems as a single discipline in
isolation. An interdisciplinary team with
professionals and family in one place at the
same time enhances communication and
decision-making in medical and school
settings. However, these teams are not
available in all settings (see Chapter 1 for
further discussion of types and functions
of teams, which in some instances may be
virtual teams). Findings from all evaluations
must be communicated clearly in meaning-
ful ways so that appropriate coordinated
recommendations can be made. Physician
input is of utmost importance as part of
the development of management plans for
many children, including those children
who on first impression may appear to
have “simple” sensorimotor or behavioral
problems related to feeding. Determination
of medical-based contributing factors pro-
vides the basis for optimal treatment. These
options likely vary according to history,
physical examination, and findings during
the clinical feeding evaluation (evaluation
and examination are used interchangeably).
Clinic/Bedside Evaluation of
Oral Feeding: Global Factors
Prefeeding Considerations
Consistent with the ICF-CY framework
(WHO, 2007), broad mealtime factors need
to be considered as a first step to adhere to
a “whole-child” approach that is critical to
optimizing feeding function. For some chil-
dren, psychotherapeutic consultation and
intervention may be needed. Behavioral
components related to feeding may involve
primary or secondary complicating factors
(Chapter 13). Children who are difficult to
feed have long been reported to be at higher
risk for abuse by caregivers and in turn may
not grow as well as children who are per-
ceived as easy to feed (e.g., Klein Stern,
1971). Thus, maladaptive habits become
established and may persist for a variety
of reasons.
Understanding physical, social, and
cultural contributions to mealtime envi-
ronments is important in order to develop
a holistic perspective of the feeding process.
Assessment is needed of the physical envi-
ronments in which children eat (e.g., home,
school, child care, restaurants, and social
gatherings). Preparation of meals and the
feeding process are ways in which families
communicate their values and priorities,
in addition to being a method of meeting
nutritional needs. Assessment must include
understanding and respect for family goals
and priorities.
Knowledge of normal developmental
patterns and the sequential advancement
of oral sensorimotor skills is a prerequisite
to understanding the assessment process
(Chapter 2). Assessment of oral sensorimo-
tor and feeding activities follows directly
from the knowledge of normal expectations
and variations from those expectations.
Professionals Involved
in Clinical Feeding/
Swallowing Evaluations
This chapter presents a comprehensive
discussion related to the clinic/bedside
evaluation of swallowing and feeding that
is usually performed by a speech-language

pathologist (SLP) and/or an occupational
therapist (OT) with specialized training in
pediatric swallowing and feeding disorders.
The background of the oral sensorimotor/
feeding specialist may vary in different set-
tings. However, the knowledge and skills of
each person must be extensive and cover
all the areas mentioned previously. The
risks for serious physiologic consequences
are great for children with swallowing and
feeding disorders if accurate diagnoses are
not made or if treatment is carried out in
fragmented ways or with inappropriate
techniques. This chapter is not intended
as a procedural manual but is designed to
describe aspects of swallowing and feed-
ing that need to be observed and evaluated.
The neonatal intensive care unit (NICU)
is a specialized environment that requires
additional knowledge and skills for profes-
sionals involved in advancing oral feeding
with these very high-risk infants. Just as
in other environments with children who
exhibit multifactorial swallowing and feed-
ing disorders, these evaluations should be
conducted in conjunction with an interdis-
ciplinary team of professionals who have the
child’s total well-being as the primary goal.
The team approach is stressed throughout
this chapter with realizations that types of
teams differ from place to place and may
even be virtual teams who communicate via
interactive distance mechanisms.
Principles of Clinic/Bedside
Feeding Evaluation
Initial evaluations may be carried out by a
clinician in a single discipline or by a team
of professionals. What do clinicians need
to know when infants and children are
brought to them for screening or evalua-
tion of oral feeding? The underlying best
evidence encompasses the following critical
areas of extensive knowledge needed:
n “typical” development from prenatal
through early months and years,
n etiologies underlying swallowing and
feeding disorders,
n differences between delayed and
disordered development, and
n interrelationships of systems (e.g.,
cardiopulmonary, upper airway,
gastrointestinal, neurologic, cranio-
facial, sensorimotor, and behavioral
interactions).
Knowledge of neurologic sensorimotor
learning principles forms a basis for inter-
pretation of observations/findings during a
“typical” feeding situation for all infants and
children. The process of skill acquisition
for advancing oral feeding appears related
to motor learning approaches per review
of school-aged children (Sheppard, 2008)
and likely for infants and young children
as well. Sheppard noted that deficiencies in
swallowing and feeding may encompass eat-
ing, saliva control, swallowing during oral
hygiene, and swallowing medications. Cli-
nicians are reminded that increases in oral
skills tend to correlate closely with global
acquisition of motor skills (Benfer et al.,
2013; Telles Macedo, 2008). A basic tenet
of motor learning is the concept of train-
ing to the task as directly as possible (Klein
Jones, 2008). Principles for habilitation/
rehabilitation after brain damage appear
salient for infants and children, although
data are limited. Neuroprotection and neu-
roplasticity are discussed in a later section
of this chapter.
Critical Thinking,
Clinical Reasoning, and
Clinical Judgment
Given the paucity of information about
feeding/swallowing development and its
disorders, providing evidence-based care

for affected children is challenging. Clini-
cians may find it helpful to consider three
interrelated concepts—critical thinking,
clinical reasoning, and clinical judgment
(Victor-Chmil, 2013).
Critical thinking refers to cognitive
processes for the analysis of information
derived from evidence and science rather
than assumptions or conjectures. When
acquiring knowledge, clinicians need to
judge the type and credibility of sources and
recognize the impact of any of their biases
or those related to the source of informa-
tion (Hayes, Chatterjee, Schwartzstein,
2017; Schwartzstein Parker, 2011). Pri-
mary sources of information appear as
original research articles in peer-reviewed
journals. When determining the utility of
these articles, clinicians are urged to con-
sider the study design, population studied,
statistical analysis, appropriateness of the
methodology, and whether the data support
the conclusions. Secondary sources include
textbooks and review articles. These sources
of information were created by authors
who interpret information from a range of
sources (e.g., primary or anecdotal). Cli-
nicians are encouraged to determine the
appropriateness of citations and presence
of potential of intentional or unintentional
author biases (Schwartzstein Parker,
2011). Consensus statements, white papers,
and credible websites (e.g., PubMed.gov
[https://www.ncbi.nlm.nih.gov/pubmed]
or Online Mendelian Inheritance in Man
[OMIM, https://www.omim.org/]) pro-
vide information from a panel of experts to
inform readers about complex information
and to guide problem-solving and decision-
making. Nonetheless, clinicians need to
determine whether the reported informa-
tion is relevant to their patient population
and to be aware of the biases and scope of
evidence reviewed by the group issuing the
report. Finally, social media and professional
and support groups have become a means
of obtaining information. Again, clinicians
are advised to consider the source of the
information reported (e.g., primary or anec-
dotal), biases, and the transparency of finan-
cial disclosures. Critical thinking is used to:
n define a patient’s problem,
n gather and analyze patient information,
n examine the evidence-based practice in
caring for the patient,
n evaluate the relevance of the informa-
tion, and
n decide on possible “discipline-specific”
actions to improve the patient’s
physiologic and psychosocial outcomes
(Connors Siner, 2015; Foundation for
Critical Thinking, n.d.; Tanner, 2006;
Victor-Chmil, 2013).
The term clinical reasoning refers to the
application of the information (derived dur-
ing the critical thinking process) to the clin-
ical situation for an individual patient. Clin-
ical reasoning requires the integration of the
“best data” for the identification of the most
appropriate interventions that will improve
the specific patient’s condition. It requires
the ability to sort through a cluster of fea-
tures presented by a patient and accurately
assign a diagnostic label, with the develop-
ment of an appropriate treatment as an end
goal (Connors Siner, 2015; Foundation
for Critical Thinking, n.d.; Tanner, 2006).
The key elements of clinical reasoning are
knowledge, skill or experience, and context
(e.g., professional or institutional wisdom
and culture) (Bowen, 2006).
Clinical judgment refers to decisions
based on “knowing the patient.” Clinical
judgments may include interpretation or
conclusion about a patient’s needs, con-
cerns, or health problems, and decision to
take (or not) actions, use or modify stan-
dard approaches, or improvise new ones
as deemed appropriate by the patient’s
responses (Tanner, 2006).

Steps and Goals of Clinical
Feeding/Swallowing
Evaluation (Figure 7–1)
n Identify possible underlying etiologies
underlying the dysphagia.
n Formulate hypotheses regarding the
nature and severity of the dysphagia.
n Establish baselines (e.g., respiratory
function and oral sensorimotor skills).
n Introduce therapeutic modifications.
n Investigate feeding options safe for
the child in the context of family and
cultural differences.
n Determine whether instrumental
swallow assessment may be needed; if
so, which instrumental assessment will
yield the necessary information.
n Assess readiness of child to participate
in an instrumental assessment.
n Develop and modify processes on the
basis of findings.
n Establish a plan for follow-up testing,
observation, and/or intervention.
Consultation received
Initial Assessment
1. Review chart and other reports
2. Get history and concerns from caregivers
3. Observe child (physical exam)
Respiration abnormal
(Airway)
Respiration normal
(Airway)
Clinical feeding and swallowing evaluation
Suspicion for
aspiration
No suspicion for
aspiration
Instrumental exam
(VFSS or FEES)
or further medical workup
Develop oral-sensorimotor plan
in context of child's global needs
1, Monitor status
2. Alter plan as needed
Airway evaluation
Hold feeds
Airway clear
Figure 7–1. Steps in clinical or bedside swallowing and feeding evaluation for infants and
children.

Criteria for Referral
Criteria for referral of children who need a
clinical feeding evaluation are variable and
may change over time (Table 7–2). Parents’
concerns are most frequently the basis for
referrals (Barkmeier-Kraemer et al., 2017;
Benfer et al., 2017). Parents often take their
concerns first to the child’s pediatrician,
who then makes a referral. However, there
are many ways and reasons for children to
be seen for a clinical feeding evaluation. The
importance of early identification of feed-
ing/swallowing problems is emphasized
as a means of minimizing the severity and
hopefully reducing the time and energy
required for attainment of optimal feeding
status. Remember that not all children will
be total oral feeders.
Screening and
Evaluation Tools
Given the paucity of information about
normal and atypical feeding/swallowing
development in general and the need to
care for these children with swallowing and
Table 7–2. Common Criteria for Referral of Infants and Children
for Swallowing and Feeding Evaluation
Sucking and swallowing incoordination
Weak suck
Breathing disruptions or apnea during feeding
Excessive gagging or recurrent coughing during feeds
New onset of feeding difficulty
Diagnosis of disorders associated with dysphagia or
undernutrition (examples in several chapters)
Weight loss or lack of weight gain for 2–3 months especially in the
first 2 years of life (undernutrition)
Severe irritability or behavior problems during feeds
History of recurrent pneumonia and feeding difficulty (Chapter 10)
Concern for possible aspiration during oral feeds
Lethargy or decreased arousal during feeds
Feeding periods longer than 30–40 min on a regular basis
Unexplained food refusal and undernutrition (better term than
failure to thrive) (Chapter 13)
Drooling persisting beyond age 5 years (Chapter 11)
Nasopharyngeal reflux with feeding
Delay in feeding developmental milestones
Children with craniofacial anomalies (Chapter 12)

feeding concerns, it is not surprising that
new tools and scales have emerged. We are
not endorsing any specific tools. Rather our
goals are for clinicians to consider assess-
ing which tools would be appropriate for the
infant or child in their care. Specific tools
fit into several broad classifications from
screening to in-depth evaluations. Selection
of the appropriate tools or scales requires
clinicians to understand how the tool was
developed and its dimensions. Regardless
of the tool or scale being considered, clini-
cians need to ask questions when selecting
clinical tools.
Screening and Screening Tools
Screening and screening tools for swallow-
ing and feeding disorders are used to dis-
tinguish between infants and children who
demonstrate problems or are at risk for
developing problems from those who are
not. Screening approaches can range from
observations of behaviors (discussed in fol-
lowing pages), to purposeful and focused
key questions, to vetted questionnaires.
Generally, screening is not time consum-
ing, and when effective, it provides infor-
mation about whether there is or is not a
suspicion of a problem. When problems are
suspected, infants and children are referred
for comprehensive clinical feeding evalu-
ation. Early identification is urged so that
the negative impact of feeding disorders can
be prevented or at least minimized. Exam-
ples of purposeful and focused key ques-
tions follow.
Purposeful Focused Key
Questions or Red Flags
Professionals who provide primary care to
infants and children are in front-line posi-
tions to be alert to potential swallowing
and feeding problems. Without requiring
significant additional time in the course of
routine examinations, a few key questions
can be asked and enable early identification
and thus early intervention before problems
become chronic. Importantly, early screen-
ing may prompt comprehensive evaluations
that hold the potential for minimizing the
negative impact on the child and family
while maximizing positive outcomes.
An example of purposeful key questions
for physicians and other professionals to ask
parents was proposed by Arvedson (2013).
Questions initially covered four important
problem areas that are associated with feed-
ing difficulties, with a fifth question related
to gastrointestinal (GI) factors added. Ques-
tions relate to respiratory concerns, pro-
longed mealtime duration, slow or lack of
adequate growth, GI retching/vomiting, and
mealtime stress (Table 7–3). “Yes” responses
to these questions provide corresponding
“Red Flags” that may lead to referral for a
comprehensive clinical feeding evaluation.
These red flag factors capture children at
high risk for swallowing and feeding diffi-
culties as well as undernutrition.
The ability of these questions to detect
the difficulties needs to be explored empiri-
cally. Benfer and colleagues (2017) com-
pleted a retrospective study to determine
the relationship between commonly cited
feeding/swallowing risk factors, includ-
ing the red flags, and outcomes of feeding/
swallowing difficulties and undernutrition
in children with cerebral palsy (CP). They
concluded that the red flags present as
feasible screening questions for parents of
children with CP, but need supplementation
with an “eating/drinking difficulty” item.
Questions remain regarding generalization
across a range of etiologies and severity of
swallowing and feeding problems. Thus,

research continues to identify sensitivity
and specificity of relevant items.
Vetted Questionnaires
Vetted questionnaires appear to fall into two
primary categories. First, questionnaires are
directed for families to use to detect pos-
sible problems in their children (e.g., Infant
and Child Feeding Questionnaire (ICFQ)
(Barkmeier-Kramer et al., 2017). Second,
questionnaires are used by medical and
health care professionals (e.g., Dysphagia
Disorder Survey [DDS]) (Sheppard, Hoch-
man, Baer, 2014).
Pediatric Clinic/Bedside
Evaluation/Assessment Tools
Little is known about clinical properties
and psychometric soundness of clinical
pediatric oral sensorimotor swallowing and
feeding assessments. Systematic reviews of
assessment tools concluded that overall,
Table 7–3. Red Flags/Key Questions to Aid in Decisions for Referral to Clinical Swallowing
and Feeding Assessment
Questions or
Concerns
Examples of Presentations With Rationale and
Literature Support
Airway/respiratory • Gurgly voice, coughing, and multiple swallows best predictors of
dysphagia (Benfer et al., 2015)
• Repeated chest infections and hospitalizations common signs
of unsafe swallowing (Peterson et al., 2006)
Feeding duration • Longer than 30 minutes frequently or 2.5 hours per day
(Sullivan et al., 2004)
• Greater than 45–60 minutes can lead to malnutrition (Hals, Ek,
Svalastog, Nilsen, 1996; Ramage, Simpson, Thomson,
Patersen, 1997)
Weight gain or lack
of weight gain
• Lack of weight gain over just 2–3 months in children less than
2 years of age like weight loss in older children and adults
• Oral sensorimotor impairment may affect functional capacity of
children and health quality of life (Liu Saltzman, 2009)
GI Retching/vomiting • Up to 77% of children undergoing PEG placement have
histories of vomiting or retching, indicative of GER (Avitsland
et al., 2006)
• PEG insertion does not lead to increased reflux in children with
CP (Kakade, Coyle, McDowell, Gillick, 2015)
Stress at mealtimes • Battles not likely to get child to eat more
• Poor feeding ability is major stress for parents (Sullivan, 2004)
• Stress may be more prominent in parents, the child, or both
Note. CP = cerebral palsy; GER = gastroesophageal reflux; PEG = percutaneous endoscopic gastrostomy.

psychometric evidence is inconsistent and
inadequate for the evaluative tools (Barton,
Bickell, Fucile, 2017; Heckathorn, Speyer,
Taylor, Cordier, 2016). These tools have
high variability in target populations, in
assessment designs, in domains of assess-
ment, and in scoring. Many assessments
do not provide instructions for scoring
or interpreting scores. Most assessments
need to be used with caution, and further
research is needed to evaluate psychometric
properties of the assessments. Examples of
tools can be found in Benfer et al. (2017);
Kamide, Hashimoto, Miyamura, and Honda
(2015); and Sellers, Pennington, Mandy,
and Morris (2014).
The need continues for data-based
research in both normal development and
disordered development in order to provide
evaluation and intervention for infants and
children with a wide range of types of feed-
ing problems/disorders and their severity.
Professionals are encouraged to learn from
the literature, to gain experience with chil-
dren and caregivers, and to work as team
members in whatever ways are possible. Pri-
mary bases for evaluation of feeding arise
from what is known about global neuro-
developmental sensorimotor learning pro-
cesses and increasingly what we are learning
about neural plasticity (see Chapter 2).
Criteria for Referral
for Clinical Evaluation
of Feeding
The earliest communication between parent
and infant occurs through feeding. Thus, a
feeding problem in the newborn period is
often perceived by parents as a significant
concern. The ramifications of abnormal
feeding, which usually involve high levels
of stress for parents as well as infant/child,
permeate all aspects of their lives. However,
typically improvement can be anticipated as
the central nervous system (CNS) matures,
even in children with severe neurologic
impairment. As infants develop increased
muscle strength and learn to make com-
pensatory movements, they are likely to
improve oral feeding skills. Nonetheless,
feeding problems may become more promi-
nent, or new problems may arise over time.
The early promotion of optimal oral sen-
sorimotor function in infants identified as a
high priority in the past (e.g., Krick Van
Duyn, 1984; Ottenbacher, Bundy, Short,
1983), continues to be a priority. Chronic
oral sensorimotor and feeding problems,
which develop during infancy, frequently
result in cycles of forced feeding and inad-
equate nutrition status. Delayed develop-
ment of sensorimotor skills can result in
prolonged feeding times and can contribute
to increased tension in the caregiver, which
in turn exacerbates feeding problems. An
oral sensorimotor and feeding assessment
for infants and children takes many vari-
ables into account. Knowledge of anatomy,
embryology, and physiology related to swal-
lowing and feeding as well as global normal/
typical development provides the founda-
tion for evaluation of swallowing and feed-
ing (Chapter 2). Professionals must keep in
mind that the complexities of neurodevel-
opment, the airway, the GI tract, nutrition,
and tone/positioning have ramifications
for a clinical oral sensorimotor and feeding
evaluation. This evaluation focuses on a
relatively narrow set of behaviors and skills
operant in the total feeding process.
Assessment and treatment programs
frequently place major emphases on oral
sensorimotor function and swallowing. As
previously reviewed, consistent with the
ICF-CY framework (WHO, 2007), broader
mealtime factors need to be considered to
adhere to a “whole child” approach, which

is critical to optimizing feeding function.
For some children, psychotherapeutic con-
sultation and intervention may be needed.
Behavioral components related to feeding
may involve primary or secondary compli-
cating factors (Chapter 13). Children who
are difficult to feed have long been reported
to be at higher risk for abuse by caregivers
and in turn may not grow as well as children
who are perceived as easy to feed (e.g., Klein
Stern, 1971). Thus, maladaptive habits
become established and may persist for a
variety of reasons.
Consideration of physical, social, and
cultural factors influencing mealtime envi-
ronments is important in order to develop
a holistic perspective of the feeding process.
Assessment is needed of the physical envi-
ronments in which children eat (e.g., home,
school, child care, restaurants, and social
gatherings). Preparation of meals and the
feeding process are ways in which families
communicate their values and priorities,
in addition to being a method of meeting
nutritional needs. Assessment must include
understanding and respect for family goals
and priorities.
Knowledge of normal developmental
patterns and the sequential advancement
of oral sensorimotor skills is a prerequisite
to understanding the assessment process
(Chapter 2). Assessment of oral sensorimo-
tor and feeding activities follows directly
from the knowledge of normal expectations
and variations from those expectations.
The knowledge and skills of each profes-
sional must be extensive and cover all the
areas mentioned previously. The risks for
serious physiologic consequences are great
for children with feeding disorders if accu-
rate diagnoses are not made or if treatment
is carried out in fragmented ways or with
inappropriate techniques. This chapter is
not intended as a procedural manual but is
designed to describe aspects of swallowing
and feeding that need to be observed and
evaluated. The NICU is a specialized envi-
ronment that requires additional knowl-
edge and skills for professionals involved in
advancing oral feeding with these very high-
risk infants (focus later in this chapter). Just
as in other environments with children who
exhibit multifactorial swallowing and feed-
ing disorders, these evaluations should be
conducted in conjunction with an interdis-
ciplinary team of professionals who have the
child’s total well-being as the primary goal
(focuses on transition feeders and beyond
will be discussed later in this chapter). The
team approach is stressed throughout this
chapter with realizations that types of teams
differ from place to place, and may even be
virtual teams who communicate via inter-
active distance mechanisms. The next sec-
tions will cover the following topics: review
of the medical, developmental, and feeding
history; physical examination; and observa-
tion of a typical meal.
Review of Family,
Medical, Developmental,
and Feeding History
The medical record is a primary source of
information and is usually readily accessi-
ble to medical professionals and hopefully
to primary caregivers and to legal guard-
ians, especially with increased access to
electronic medical records in recent years.
When professionals in educational settings
do not have easy access to records, they may
gain information primarily from interview-
ing parents and other caregivers. Family,
prenatal, birth, and neonatal histories are all
important. History should encompass the
range of information suggested in Appen-
dix 7–A, which has detailed sections for
history, physical examination, and feeding

observation that is likely to be particularly
helpful for professionals with limited expe-
rience in this area of high-risk patient care.
Whenever possible, the clinician should get
information directly from a primary source,
rather than relying on other interpretations
that may be subject to error. In the presence
of conflicting or incomplete information,
each clinician should obtain history from
reliable sources.
Family and Social History
Primary caregivers are critical to the care of
each child. It is important to know all who
live in the environment with the child. The
social history information aids in decision-
making for a management plan. Some chil-
dren may be living in a rapidly changing
social situation. Cultural, educational, and
socioeconomic factors are likely to impact
management decisions.
Family history may reveal similar prob-
lems in other family members. These prob-
lems may include, but are not limited to,
neurologic deficits, cleft palate or other
craniofacial anomalies, respiratory/breath-
ing factors, and feeding difficulties. Envi-
ronmental factors likely to have an adverse
effect on a child’s respiratory status include
smoking or pets in the home. Secondhand
smoke can have a deleterious effect on
infants and children with underlying pul-
monary deficits (e.g., Mason, 2016; Torok,
Winickoff, McMillen, Klein, Wilson,
2017). Even when caregivers smoke out-
side the home, negative effects may occur
because of the lingering effects on skin
and clothes. Daycare exposes infants and
young children who are born preterm and
exhibit underlying chronic lung disease to
an increased risk of respiratory morbidities
(McGrath-Morrow et al., 2010).
Medical and Developmental
Prenatal, Birth, and
Perinatal History
Prenatal History to Birth
Prenatal history factors relevant to swallow-
ing and feeding include, but are not limited
to, maternal infection, medications dur-
ing pregnancy, substance abuse by father
or mother, radiation exposure, toxemia,
bleeding, thyroid disease, or polyhydram-
nios (excessive volume of amniotic fluid),
to name a few. Helpful information about
the birth and perinatal period may include,
but is not limited to, Apgar scores, cord
pH, trauma during delivery, prolonged
hypoxia or anoxia, intubation, other respi-
ratory distress, surfactant therapy, continu-
ous positive airway pressure (CPAP), high
flow nasal cannula (HFNC), and cardiac
status.
The Apgar scale (Apgar, 1966; Com-
mittee Opinion, 2015) continues to be used
routinely as part of the immediate care of a
newborn infant. This scale permits a quick
and thorough examination of a neonate’s
response to the birth process and imme-
diate adaptation to extrauterine life. Five
characteristics are measured: heart rate,
respiratory effort, muscle tone, reflex irri-
tability, and color (Table 7–4). Each attri-
bute is scored 0, 1, or 2, and those scores
are summed for a maximum score of 10.
Assessment is done routinely at 1 min after
birth and again at 5-min intervals thereaf-
ter for an infant with scores lower than 7
(Committee Opinion No. 644, 2015). Each
assessment takes about 1 min to complete.
Scores are usually interpreted as poor (0–3),
fair (4–7), and good (7–10).
Apgar scores are frequently used as
a control for the population of infants
investigated in neonatal follow-up studies,

although a direct association has not been
established between any specific complica-
tion during the birth process and a bad neu-
rologic outcome in term newborns (Nelson
Ellenberg, 1984). While a 1-min APGAR
score between 0 and 3 is not predicative of
future neurologic dysfunction, infants with
a low 5-min Apgar score (less than 7.0) have
greater risk for mortality and morbidities
associated with prematurity (Committee
Opinion No. 644, 2015; Juretschke, 2000;
Phalen, Kirkby, Dysart, 2012).
Cord blood testing can be used to
evaluate a newborn’s health. Cord blood is
obtained right after birth from the umbili-
cal cord that is clamped and cut. A second
clamp is placed 8 to 10 inches (20–25 cm)
away from the first clamp. That section
between the clamps is cut and a blood
sample is collected into a specimen tube.
Measures obtained include the following:
bilirubin level; blood culture if infection
is suspected; blood gases that include oxy-
gen, carbon dioxide, and pH levels (low
pH less than 7.04–7.10); blood sugar level;
blood type and Rh; complete blood count
(CBC); and platelet count (Gomella
Cunningham, 2013). Normal values mean
that all items checked are within normal
range. Abnormal results differ depending
on the specific measure (Table 7–5). In
some instances, cord blood can be banked
or donated at the time of delivery (ACOG,
2015). Cord blood can be used to treat cer-
tain types of bone-marrow-related cancers.
Cord blood may be banked and saved for
future medical purposes (Greco Elkins,
2017; Waldorf, 2017).
A pH of 7.25 or greater has been shown
to correlate (with 92% accuracy) with a
2-min Apgar score of 7 or greater. A pH
of less than 7.15 correlates with a 2-min
Apgar score of less than 7 (80% accuracy).
Standard protocol uses the following results
(Gomella Cunningham, 2013):
n pH 7.25: Normal result; fetus is
probably normal.
n pH 7.20: Abnormal result; fetus is
acidotic. If this result occurs in the
absence of maternal acidosis, and a
repeat test done 10 min after the first
Table 7–4. Apgar Scoring System for Standardized Assessment Following Birth
Attribute
Score
0 1 2
Heart rate Absent Below 100 Above 100
Respiratory effort Absent Slow, irregular,
hypoventilation
Steady, good cry
Muscle tone Flaccid Some flexion of
arms and legs
Good flexion,
active motion
Irritability No response Some motion; cry Vigorous cry
Color Blue or pale Blue hands and
feet; pink body
Pink overall
Note. Adapted from “The Newborn (APGAR) Scoring System: Reflections and Advice,” by V.
Apgar, 1966, Pediatric Clinics of North America, 13, p. 645.

reveals the same or more acidotic pH,
delivery is indicated.
n pH between 7.20 and 7.25: Test should
be repeated. Decisions regarding
delivery depend on the clinical
situation.
Table 7–5 shows utility of these mea-
sures for understanding conditions in the
NICU. Perinatal asphyxia exists when an
antepartum event, labor, or a birth pro-
cess diminishes the oxygen supply to
the fetus. The diminished oxygen supply
causes decreased fetal or newborn heart
rate, resulting in impairment of exchange
of respiratory gases, oxygen, and carbon
dioxide and inadequate perfusion of the
tissues and major organs. Incidence figures
vary because of nonuniform clinical criteria
on which definitions are based. Incidence
of perinatal asphyxia is reported in most
centers at about 5 to 10 per 1,000 births
(McGuire, 2007). It occurs in about 9% of
infants less than 36 weeks’ gestational age
and in 0.5% of infants more than 36 weeks’
gestational age (Snyder Cloherty, 1998).
Interestingly, less than 10% of children with
CP show evidence of perinatal asphyxia.
Perinatal asphyxia is diagnosed by four
clinical criteria (American Academy of
Pediatrics, American College of Obstetri-
cians and Gynecologists, 2017; Morales et
al., 2011): (a) profound metabolic or mixed
acidemia (pH 7.00) on umbilical cord
arterial blood sample: (b) persistence of
Apgar score of 0 to 3 for greater than 5 min;
(c) clinical neurologic sequelae in the im-
mediate neonatal period to include seizures,
hypotonia, coma, or hypoxic-ischemic
encephalopathy; and (d) evidence of mul-
tiple organ system failure in the immediate
neonatal period that may include circula-
tory, digestive, and respiratory systems.
No one factor is likely to correlate with a
swallowing and feeding problem, but each
one may aid in delineation of the problem.
However, these parameters have no predic-
tive value for long-term neurologic injury
after mild to moderate asphyxia (Leuthner
Das, 2004).
Medical and Developmental
History Specific to Neonates
(First 28 Days)
Significant events pointing to neurologic
dysfunction during the neonatal period
include the need for prolonged resuscitation,
altered states of consciousness, seizures,
deficient movement, and disturbances of
sucking and swallowing (Fenichel, 2006).
Feeding deficits in these first few weeks of
life may be markers for possible underly-
ing neurologic problems (Wolthuis-Stigter
et al., 2017). Because neurologic problems
frequently manifest themselves in the neo-
Table 7–5. Abnormal Cord Blood Tests and Potential Meaning of Results
Abnormal Result Potential Meaning of Results
Low pH (less than 7.04–7.10) Higher levels of acid in blood
Positive blood culture for bacteria Blood infection
High-level blood sugar (glucose) Mother may have diabetes
Infant monitored for hypoglycemia
High-level bilirubin Many causes—could be due to infections

natal period, information is needed regard-
ing arousal and alertness, respiratory status,
rooting and other reflexes (see Chapter 3),
medication, and nonnutritive sucking, as
well as prior medical tests and any surgeries.
Feeding History
Feeding history likely differs from one
reporter to another because perceptions
vary about the child’s skills and abilities.
Children commonly are variable in their
behaviors among feeders and environments.
Variability is to be expected and often com-
plicates decision-making for intervention
options. The descriptions of feeding behav-
iors are usually accurate and reliable from
each person’s perspective. Nonetheless,
clinicians must sort out these inconsistent
reports in order to define the underlying
feeding disorder to whatever extent pos-
sible. For example, a child who refuses
food or is markedly picky/finicky may be
perceived as lazy or not hungry when, in
fact, the child is saying “no” because of an
underlying physiologic problem resulting
in discomfort or pain that could be related
to gastroesophageal reflux or eosinophilic
esophagitis, to give a couple of examples
among many other possibilities.
When asked about concern for aspira-
tion, caregivers of 16 of 48 children who
demonstrated aspiration on fluoroscopy
said they had no concern regarding possible
aspiration because the child did not cough
or gag when eating and drinking (Arved-
son, Rogers, Buck, Smart, Msall, 1994).
Videofluoroscopic swallow study (VFSS)
revealed silent aspiration for all but two
(94%) of the children who aspirated in this
retrospective review. This finding empha-
sizes that with CNS damage, the probabil-
ity of a cough response to aspiration is low.
Caregivers cannot accept the lack of cough
as a sign of safe feeding. Other signs and
symptoms of respiratory-related concerns
that may become evident by history include
hoarseness, gurgly voice quality, inspiratory
stridor, recurrent pneumonias or chronic or
long-lasting upper respiratory tract infec-
tions, apnea, and cyanosis (Chapter 4) (e.g.,
Illingworth, 1969; Kramer, 1985).
Clinicians are wise to get information
from more than one person and in multiple
formats. Caregivers may complete a printed
questionnaire in advance of the initial clinic
evaluation. Clinicians can ask questions in
an interview during the evaluation session.
A combination of questionnaire and inter-
view is likely to yield the most complete and
comprehensive history. Ensuring that the
questions asked are clear and understand-
able requires knowledge of the caregivers’
language, culture, and education.
Parents should be asked to describe
feeding behaviors rather than answer sim-
ple questions that require “yes” or “no” re-
sponses. For example, “Describe what your
baby does when the milk flow is too fast,”
gives more information than, “Does your
baby choke when the milk flow is too fast?”
The answer to “Tell me how your child
chews” yields more useful information to
the clinician than a simple question, such
as, “Does she have trouble chewing?”
Feeding history includes questions
relative to the factors shown in Table 7–6.
These items are applicable even for infants
and children who have never fed orally.
(Appendix 7–A shows options.) Duration
of mealtimes should be explored thor-
oughly. Lengthy mealtimes can be a marker
for swallowing and feeding problems (e.g.,
Hals, Ek, Svalastog, Nilsen, 1996; Korth
Rendell, 2015; Ramage, Simpson, Thom-
son, Patersen, 1997; Sullivan et al., 2004).
Longer feeding times do not compensate for
the severity of feeding impairments in many

children with multiple disabilities (Gisel
Patrick, 1998). Mealtimes should take
approximately 30 min in most cultures. If
on a routine basis 45 to 50 min or more are
required to complete a meal, changes need
to be made to improve the efficiency. The
risk for aspiration increases with the dura-
tion of mealtimes (Arvedson et al., 1994).
Duration of mealtimes also must be consid-
ered in relationship to other activities that
are important in each day. The child should
not expend more energy eating than what
is consumed. Some mothers have reported
spending up to 7 hours a day feeding a
child (Johnson Deitz, 1985). Types of
food refusals are noted (e.g., turning head,
throwing food, expelling/spitting food out
of the mouth, leaving the table). Clinicians
should inquire about more examples or
descriptions of stress involved in mealtimes.
Other considerations include, but are not
limited to, religious and/or cultural factors
that affect family food choices as well as
mealtime habits.
The nutrition status (Chapter 6) and the
interactive behaviors of the caregiver and
childarealsoimportantfactors(Chapter13).
The long-term prognosis for development
of functional oral sensorimotor skills and
safe oral feeding relates directly to the under-
lying health and neurologic status. The infor-
mation gained through thorough history
Table 7–6. Factors Included in a Feeding History for Oral and Nonoral Feeders
Position(s) for feeding and seating arrangements
Duration of feeding times (average and range)
Intervals between feedings or meal times (from start of one feeding to start of next)
Tube feeding (type, partial or total nutrition, nighttime rate if overnight feeds)
Infants: Breast- or bottle-feeding (types of nipples, formula)
Infants burping: Spontaneous? Feeder interrupt to burp?
Children who get food as well as liquid: Types of textures, use of utensils
Child’s participation in self-feeding process (total or assisted)
Diet: At least a 3-day diet history is helpful including all food and liquid with
amounts; permits dietitian to calculate nutritional value and calories
Respiratory status: Aspiration pneumonia, bronchitis, asthma, etc. Noisy
breathing, gurgly voice quality with feeding, coughing, choking
Other signs of distress: Fussy during feeding, food refusal, falling asleep,
arching, neck hyperextension
Other factors: Tests (e.g., upper gastrointestinal study [UGI], esophageal
manometry, endoscopy, scintiscan, pH study, videofluoroscopic swallow
study [VFSS]; flexible endoscopic examination of swallowing (FEES), surgical
procedures, medical treatments, medications)
Sleep patterns: Restless, waking during the night, snoring, mouth breathing
Cognitive and communication status: Verbal and nonverbal skill levels
Behavior during meals: Stress at mealtimes, refusals, participation with family
History of therapeutic intervention for developmental or feeding problems

taking is invaluable in planning the rest of
the evaluation. There is no single pediatric
assessmentscalethatcanberecommendedto
encompass all aspects of a clinic swallowing
and feeding evaluation. In some instances,
clinicians may take portions of commercially
available scales and modify them to meet
the needs of their populations, institutions,
or practice patterns. Standardized processes
with reliability and validity are urged as aids
in data collection for research and clinic pur-
poses to provide practice guidelines across
institutions and populations.
(Prefeeding Assessment)
The clinical examination of swallowing
and feeding for all infants and children
begins with overall observation of the “at
rest” posture and position. The observer
realizes that underlying tone and strength
are particularly important as a basis for
decision-making regarding oral feeding
safety. It is important for all professionals
to do a lot of looking and listening before
focusing on the mouth and feeding. The
initial signs and symptoms of feeding dif-
ficulties may be markers for broader cen-
tral or peripheral nervous system deficits
and closely related to airway and GI tract
function. During prefeeding observations,
clinicians note deviations from “normal”
expectations, even though normative data
are lacking in many aspects of feeding (e.g.,
Arvedson, 2008; Arvedson Rogers, 1993;
Korth Rendell, 2015; Marcus Breton,
2013). Observations should focus on:
n interactions between parents/caregivers
and child;
n posture, position, tone, and movement
patterns, particularly head, neck, and
trunk;
n respiratory patterns (e.g., mouth
breathing to compensate for problem
with nasal breathing, effort [retractions
suprasternal and/or substernal, inspira-
tory stridor as sign of upper airway
obstruction], alterations in rate that
may interfere with feeding or represent
instability);
n overall responsiveness, temperament,
affect;
n alertness, ability to sustain attention to
task;
n response to sensory input (e.g., vestib-
ular, proprioceptive, visual, olfactory,
tactile, auditory); and
n signs of self-regulation, self-calming.
Clinicians must be able to interpret cues
from the child indicating readiness to feed
or not to feed as the case may be.
The overall goals of this assessment are
to determine the nature of the problem
and best possible options for management.
Assessment is not a one-time event but an
ongoing process with caregivers always inte-
gral to both assessment and treatment.
Readiness for Feeding:
Sensorimotor and
Posture Factors
Oral Sensorimotor Assessment
Sensory and motor functions are inter-
twined and must be considered in light of
cranial nerve innervation to the muscles
involved in oral and pharyngeal phases of
swallowing. The cranial nerves that inner-
vate muscles for swallowing all provide
sensory and motor input, except CN XII,
which provides motor control to the intrin-
sic muscles of the tongue (see Chapter 2). As
a result of developmental processes, older
infants and children acquire competence in
discernment of the physical characteristics

of food so that they ingest it voluntarily
and safely. Bosma (1986) in his seminal
research stated that sensory information is
generated primarily by voluntary motions
of the tongue, lips, and mandible. General
responses to visual, auditory, olfactory, tac-
tile, vestibular, and proprioceptive stimuli
are observed before approaching the mouth
to observe responses to taste. Stimuli can be
varied on several parameters, for example,
degree of brightness, loudness of sounds,
and firm-to-light touch to the face and
mouth. Beware that light touch may be tick-
lish, which is usually a negative experience.
The basic guideline is “firm but gentle” usu-
ally in rhythmic stroking motions at one
stroke per second from periphery to central
structures.
Sensory processing involves alerting to,
interpreting, and organizing sensory input
to fully participate in a healthy and pleas-
ant mealtime experience (Fraker, Fishbein,
Cox, Walbert, 2007). Zobel-Lachiusa and
colleagues (2015) administered standard-
ized measures of sensory differences and
eating behaviors to children with autism
spectrum disorder (ASD) and peers with
typical development. They noted that the
children with ASD scored significantly
differently from the children with typical
development on both sensory differences
and eating behaviors. Oral sensorimotor
intervention strategies are also used with
children with neurologic disorders (Gisel
et al., 2003; Gisel, 2008; Snider, Majnemer,
Darsaklis, 2011). These findings empha-
size the importance of gathering informa-
tion regarding a child’s sensory processing
during a caregiver interview as well as a
mealtime observation.
Oral sensory assessments for infants
and children are not likely carried out in a
standardized method. However, astute clini-
cians describe their observations in objec-
tive terms so that changes over time can be
documented for measurable gains when
they occur. Sensory histories include test
items that assess oral sensory defensiveness.
The Sensory Profile II evaluation includes
a section on oral sensory/feeding (Dunn,
2014). Astute clinicians obtain information
through a feeding observation. It may be
challenging to differentiate a motor versus a
sensory response during feeding. Table 7–7
provides guidelines to interpreting a child’s
responses during a feeding observation.
Clinicians evaluate the global mealtime
sensory environment as well as a child’s
responses to specific oral sensorimotor
input. An evaluation of the mealtime sen-
sory environment can assist with identify-
ing strengths and challenges that contrib-
ute to a child’s ability to focus on eating.
These observations may provide informa-
tion regarding a child’s food choices and
refusals. Children use information from all
senses while eating that include vestibular,
proprioceptive, visual, olfactory, tactile,
gustatory, and auditory input (Fraker et al.,
2007; Korth Rendell, 2015; Morris
Klein, 2000).
The vestibular system gives children an
understanding of their position in space and
it alerts to movement. A poorly positioned
child may be distracted from the meal
because of extraneous movement. Please
refer to the section on posture and seating
for further discussion.
The proprioceptive system alerts indi-
viduals to input from muscles, tendons,
and joints. The system is activated with
stretching of joints or resistance against
muscle contraction. For instance, notable
proprioceptive input is elicited as a child
chews crunchy foods or high-density foods
because the bolus provides resistance to the
temporomandibular joint during chewing.
Children also receive proprioceptive input
from their tongue and lips as they suck liquid
from a straw. Proprioception offers informa-

tion regarding the density, size, and shape of
food, and therefore affects the strength and
coordination of chewing and sucking.
Visual input regarding the color, portion
size, and shape of food may affect a child’s
food choices. Some children are visually dis-
tracted in a busy school cafeteria. Children
who refuse foods may respond to olfactory
input and avoid foods with a strong smell.
The tactile system includes receptors
under our skin. Tactile input has a dual
purpose: (a) to alert us to danger and (b) to
assist us in discriminating touch input. The
tactile system alerts us to food textures. It
also assists in discriminating light touch
from deep touch. Tactile and proprioceptive
inputs combine to inform children about
the size, shape, and density of food.
A child’s response to auditory input
affects the ability to focus on mealtime in
a busy school cafeteria. Some children may
react in a negative manner to the auditory
input provided when chewing crunchy food.
The following questions can guide a
clinician evaluating a child’s responses to
environmental global sensory input during
Table 7–7. Characteristics to Aid in Differentiation of Children With Primarily Oral Sensory
Versus Primarily Oral Motor Disorders
Primarily Sensory Disorder Primarily Motor Disorder
Demonstrates nipple confusion with breast-
and bottle-feeding
Inefficient suck with breast and bottle
Inability to differentiate different tastes in a
bottle despite an intact suck
Differentiates tastes in a bottle
Manages liquids better than solid foods Oral inefficiency or incoordination is noted
with all textures
Able to sort food in a mixed texture Swallows food whole when offered mixed
textures
Holds food under tongue or in cheek and
avoids swallowing
Unable to hold and manipulate bolus on
tongue; food falls out of mouth or into
cheeks
Vomiting only certain textures Vomiting is not texture specific
Gags when food approaches or touches lip Gags after food is moved through oral
cavity
Hypersensitive gag with solids; normal
liquid swallow
Gags with liquids and solids after
pharyngeal swallow is initiated
Tolerates own fingers in mouth, does not
accept someone else’s fingers
Tolerates others’ fingers in mouth
Does not mouth toys Accepts teething toys but is unable to bite
them or maintain them in the mouth
Refuses toothbrushing Accepts toothbrushing
Note. Adapted from “Assessment and treatment of sensory motor-based feeding problems in very young
children,” by M. M. Palmer and M. B. Heyman, 1993, Infants and Young Children, 6, pp. 67–73.

a mealtime assessment (e.g., Fraker et al.,
2007; Korth Rendell, 2015; Morris
Klein, 2000):
1. Is the child distracted by inappropriate
vestibular (movement) input while
getting in and out of a chair or while
seated in a chair that offers insuf-
ficient support?
2. Does the child prefer crunchy or
chewy foods that could indicate
preference for strong proprioceptive
input?
3. Does the child attend to visual distrac-
tion inappropriately in the room?
4. Does the child comment verbally on
the odors of food or avoid sitting at
the same table in school or at home
when others are eating foods with
strong olfactory input?
5. Is the child distracted by tactile input
when touching foods or show a prefer-
ence for foods with a specific tactile
quality (e.g., lumpy, smooth, or solid)?
6. Does the child overattend to extra
neous
auditory input in the environment
(e.g., conversation, restaurant noises,
etc.)?
7. Does the child prefer savory or sweet
gustatory input?
Responses to Sensory Input
Children have long been noted to demon-
strate excessive or diminished responses
to sensory input that may have a negative
impact on their mealtime experience. Ter-
minology varies in the literature. For the
purposes of this chapter, hyposensitive and
hypersensitive responses are described as
follows.
Responses include hyposensitivity/hy-
poreactivity and/or hypersensitivity/hyper-
reactivity (Morris Klein, 1987, 2000;
Palmer Heyman, 1993). The Diagnostic
and Statistical Manual of Mental Disorders
(5th edition, DSM-5, APA, 2013) includes
sensory hyper- and hyporeactivity in the
diagnostic classification for autism spec-
trum disorder (ASD). These disorders rep-
resent problems with sensory modulation
or the ability to receive and grade sensory
input from the environment (Morris
Klein, 2000; Parham Mailloux, 2015).
Hyposensitive Responses. Children with
hyposensitive responses to oral input may
have diminished response to taste, tempera-
ture, or the proprioceptive input associated
with chewing and sucking (Arvedson
Brodsky, 2002; Fraker et al., 2007; Morris
Klein, 2000). These children may have
cravings for foods that provide increased
oral input (e.g., strong flavors, crunchy
textures, extreme temperatures), drooling,
and an inclination to stuff too much food
into the mouth. They may not notice that
there is food on their face. They may also
demonstrate poor sucking or chewing skills
because they are not receiving appropriate
sensory input to support refined skills.
Hypersensitive/Hyperreactive Responses.
There are multiple etiologies that may result
in an extreme or hypersensitive response to
oral input. Children may have a CNS disor-
der, for example, cerebral palsy. They may
show excessive responses to taste, tempera-
ture, and touch in and around the oral region
that manifests as hypertonicity or abnormal
motor movements. For example, a response
to a sour taste may be a jaw or tongue thrust.
Oral hypersensitivity (hyperreactivity) may
be a prominent symptom in children with
respiratory difficulties, esophagitis, eosino-
philic esophagitis, or gastroesophageal reflux
disease/extra-esophageal reflux disease
(GERD/EERD) (see Chapter 5). Children

who have had aversive medical treatments
that required prolonged intubation or fre-
quent suctioning also may have an exces-
sive response to food and oral experiences.
Other children may have sensory problems
due to CNS impairment that influence the
sensory innervation to the oral region (Rog-
ers Senn, 2008). In some instances, the
underlying physiologic disturbances may
be characterized as a marked oral aversion,
which is in fact to be expected and is not just
a “behavioral aversion.” Children who are
irritable during mealtime or who frequently
refuse various tastes and textures of food
may be responding to what they perceive
as aversive sensory input. Accurate diag-
noses are critical as a basis for appropriate
intervention.
Oral Sensory Defensiveness. Sensory
processing disorders may result in overre-
active response to sensory input that can
involve the whole body or be localized to
specific areas of the body (e.g., oral struc-
tures). Affected children may demonstrate a
“fight-or-flight” protective response to sen-
sory input (Korth Rendell, 2015; Marcus
Breton, 2013; Morris Klein, 2000; Stein,
Polido, Cermak, 2012). Children with
sensory defensiveness typically avoid many
textures and tastes, demonstrate aversion to
toothbrushing, and do not mouth toys. They
may put their own fingers to their mouth
but resist oral input from another individual
(Palmer Heyman, 1993). Many of these
children have no identifiable underlying
diagnosis, which makes the evaluation (and
intervention) process challenging.
Primarily Sensory or Primarily
Motor-Based Disorder?
Many children with neuromuscular dis-
orders have sensorimotor dysfunction. It
is useful to consider interrelationships of
sensory and motor systems, thus the term
sensorimotor is preferred in a generic sense.
Children are more likely to have some
degree of both sensory and motor deficits,
although it is possible that some children
may show primarily sensory or primarily
motor problems (see Table 7–7). Clinicians
are urged to consider the whole child, real-
izing that all systems interrelate in varying
degrees. Children who have a history of
invasive oral treatments related to condi-
tions such as chronic lung disease, tracheo-
esophageal fistula, esophageal atresia, and
cardiac defects also may demonstrate sen-
sorimotor dysfunction (Harding, Faiman,
Wright, 2010; Marcus Breton, 2013;
Palmer Heyman, 1993).
Some children with an abnormal re-
sponse to sensory input during mealtimes
have behavior problems related to eating
and drinking (see Chapter 13). The child’s
ability to self-calm and regulate is noted.
Adults may misinterpret these responses as
negative or belligerent behaviors. Children
with sensory problems also may learn to
manipulate the mealtime environment to
avoid unpleasant sensory experiences (see
Chapter 13 for detailed discussion).
Postural Considerations
Clinicians should focus on a child’s overall
muscle tone, motor control, postural con-
trol, and overall fine and gross motor skill
levels prior to oral feeding. Careful obser-
vations are critical before presentation of
any food and/or liquid, regardless of the
age and developmental levels of the child.
The importance of good posture for safe
and efficient feeding is well documented
(Gisel et al., 2003; Howe Wang, 2013;
Korth Rendell, 2015; Larnett Ekberg,
1995; Morris Klein, 2000; Morton, Bonas,

Fourie, Minford, 1993; Sheppard, 2008;
Snider, Majnemer, Darsaklis, 2011).
When evaluating a child’s posture dur-
ing mealtime, it is important to assess the
personal characteristics of the child, the
mealtime activity the child is engaged in,
as well as the environment (Sheppard 2008;
WHO, CF-CY 2007; WHO, ICF, 2001). Cli-
nicians examine all of these factors when
considering a child’s ability to self-feed
as well as when considering a child who
requires assistance for feeding. Examination
of physiological factors that include muscle
tone and possible structural postural defi-
cits is basic to consideration of the mealtime
environment. The mealtime environment
includes multiple factors, e.g., where the
child is eating, seating system, auditory and
visual distractions, as well as the broader
aspects of social and cultural contexts.
An example of a comprehensive evalu-
ation of posture and the mealtime environ-
ment during a self-feeding task is as follows:
A child is identified with hypertonia in bilat-
eral upper extremities and a contracture in
the elbow joint resulting in a fixed defor-
mity. The child is observed while attempt-
ing to scoop pureed food from a large bowl
with a standard metal spoon in the school
cafeteria during her 30-min lunch period.
This child is not likely to manage that task
without some adaptations. During an evalu-
ation of self-feeding, a clinician may modify
the task or the environment by offering var-
ied adaptive spoons, plates, and cups. The
child will be observed while self-feeding in
a small group versus a large group setting.
These observations form a starting point
for development of a specific plan for pos-
ture/seating, the use of adaptive devices for
self-feeding, the best textures for scooping
food, and the most motivating social envi-
ronment to promote independence and
pleasurable eating experiences.
Evaluation of
Physiological Factors
Physiologicalfactorsthatneedtobeevaluated
as part of a swallowing and feeding assess-
ment include musculoskeletal structures,
muscle tone, strength, posture, and over-
all gross and fine motor skills. Benfer and
colleagues (2013) studied children with cere-
bral palsy at Levels I to V on the Gross Motor
Function Classification System. They used
two standardized measures of oropharyn-
geal dysphagia and noted that gross motor
function levels have a stepwise relationship
withtheseverityoforalpharyngealdysphagia.
Extensive knowledge of normal develop-
ment and muscle tone is needed in order for
clinicians to make observations regarding
postural control and abnormal muscle tone
during the clinical evaluation of swallow-
ing and feeding. Posture involves the spatial
relationship of body parts to each other and
to other objects in the environment. Muscle
tone is a resting state that defines a child’s
readiness for movement and the amount
of resistance a muscle exhibits with passive
elongation.
Children with CNS disorders may dem-
onstrate abnormal muscle tone including
hypotonia or low muscle tone (Johnson,
Hoon, Kaufman, 2008). Hypotonia is
characterized by decreased resistance to
passive range of motion, increased joint
range of motion, and poor ability to move
up against gravity (Figure 7–2). Postural
differences that may be noted in a child
with hypotonia that can affect oral senso-
rimotor skills and swallowing include head,
neck, and trunk asymmetry as well as neck
hyperextension with swallowing. Other
children with CNS disorders demonstrate
hypertonic muscle tone or spasticity (Fig-
ure 7–3). Increased resistance to passive
joint movement and limitations in joint

range of motion are identified. Children with
spasticity also may demonstrate abnormal
movement patterns and remnants of primi-
tive postural reflexes. These children may
develop joint deformities that include con-
tractures. Children with athetosis demon-
strate fluctuating muscle tone that may result
in uncontrolled movements (Locke, 2008).
Based on the intricate relationships
between overall body posture and oral sen-
sorimotor function, children with CNS dis-
orders and abnormal muscle tone are at risk
for oral pharyngeal dysphagia (Benfer et al.,
2013). Evaluators therefore need to assess
tone and posture to develop a plan to opti-
mize oral sensorimotor function.
Examination of Children
(Beyond Infancy)
Prefeeding Observations
of Children
Problems with postural alignment, tone,
and positioning relate directly to the oral
sensorimotor system. Observations are
made about head, trunk, and pelvic pos-
ture with a child in the usual sitting posi-
tion in whatever seating system is used
for feeding. Particular attention should
be paid to the child’s posture to correlate
Figure 7–2. Child with decreased muscle
tone (hypotonia), hyperextended neck, and
trunk shifted to the left. She has difficulty in
self-feeding.
Figure 7–3. Child with cerebral palsy who
has increased muscle tone (hypertonia) with
increased flexion in upper extremities. She
has difficulty in self-feeding and needs pos-
tural support.

with movement patterns. The presence of
primitive reflexes, overall levels of physi-
cal activity, and any type of independent
oral stimulation are noted (e.g., putting
objects or fingers into the mouth). Clini-
cians observe the child for overall affect,
temperament, and responsiveness includ-
ing interaction with parents or other famil-
iar caregivers. Areas to evaluate include the
level of alertness, trunk, head, shoulders,
and mouth position, presence of drooling,
and means of both verbal and nonverbal
communication between parents and child.
The clinician notes the child’s use of eye
contact, head turning, touch, and avoidance
responses.
Posture influences the entire swallowing
process, not just bolus formation. Gross and
fine motor skill development, respiration
and phonation, and oral sensorimotor/feed-
ing skills are interrelated in intricate ways as
discussed in Chapter 2.
Posture and Oral Function
for Bolus Formation
A child with low muscle tone, who has dif-
ficulty moving the head up against gravity,
may demonstrate an extreme neck hyper-
extension posture as noted in Figure 7–2.
Abnormal muscle tone may also contribute
to a kyphotic trunk posture. This posture
may affect the child’s ability to orient to the
bolus efficiently. This hyperextension does
not provide a stable base or good alignment
for optimal oral sensorimotor control. Pos-
tural deficits may contribute to oral senso-
rimotor problems including tongue retrac-
tion and a tonic bite (Korth Rendell,
2015; Morris Klein, 2000). Instability
at the trunk and neck may affect a child’s
ability to have a stable jaw for oral motor
coordination.
Posture and Pharyngeal
Function for Swallowing
Neck hyperextension influences the align-
ment of pharyngeal structures and places
children at higher risk for aspiration dur-
ing oral feeding, especially with thin liquid.
Postural control also is related to coordina-
tion between respiration and swallowing
during feeding. A child must demonstrate
stability through the spine and mobility in
the rib cage for efficient respiration. This
relationship of stability and mobility is cru-
cial during the intricate coordination of
breathing and swallowing that takes place
during oral feeding.
Posture and Esophageal
Function During Swallowing
GERD/EERD may be influenced by body
posture. Lightdale, Gremse, and Section on
Gastroenterology, Hepatology, and Nutri-
tion (2013) note that healthy infants ben-
efit from fully upright positioning after a
feeding to prevent reflux. Corvaglia and
colleagues (2007) used impedance and pH
monitoring to note that premature infants
in prone or left lateral position have less
reflux than in the flat supine position. How-
ever, the prone position is strongly contra-
indicated for infants through 12 months
of age per recommendations for sleep in
SIDS prevention by the American Acad-
emy of Pediatrics (AAP) (2016). Infants
may be in prone position when they are
awake and closely supervised. Infants do
need experience in prone position to pro-
mote head lifting and upper body strength
overall (Dudek-Shriber Zelazny, 2007;
Lightdale et al., 2013). Jung and colleagues
(2012) reported that upright positioning
may reduce the frequency of reflux-related

respiratory problems but not the frequency
of reflux. Clinicians are advised to keep
abreast of the evolving evidence and AAP
guidelines regarding infant positioning.
The 2016 guidelines review positioning for
infants with specific conditions that com-
promise protection of the airway. Manage-
ment of GERD/EERD is covered in greater
detail in Chapter 5.
Optimal Feeding Posture
General guidelines for “optimal” sitting are
listed as follows. That said, optimal sitting
posture to support oral sensorimotor func-
tion must be considered for each individual
child (Alexander, 1987; Arvedson Brod-
sky, 2002) (Figure 7–4).
Optimal posture for eating/drinking:
n Neutral head position (symmetry,
midline stability), with balance between
flexion and extension
n Neck elongation, but not hyperextension
n Symmetrical shoulder girdle stability
and depression
n Symmetrical trunk elongation
n Pelvis stability, with the child’s hips
symmetrical in neutral position
n Hips, knees, and ankles each at 90°
with neutral base of abduction and
rotation
n Symmetrical and stable positioning
of the feet in neutral with slight
dorsiflexion (never plantar flexed),
supported by a firm surface
Complications arise when children have
significant scoliosis or kyphosis, making it
more difficult to achieve an optimal posi-
tion. Although this information is useful
and makes intuitive and clinical sense, it is
not based on empiric data. Further research
is needed to provide more specific guide-
lines regarding the best posture for opti-
mal oral sensorimotor function. There is
no one single best posture for all children.
Variations are needed and will continue to
be needed to meet the needs of individual
infants and children.
Evaluation of Infants in the
Neonatal Intensive Care Unit
All professionals involved in the care of
infants in the NICU focused on facilitating
oral feeding must have extensive knowl-
edge about the physiology of sucking and
swallowing as well as etiologies and under-
lying diagnoses of the infants. It is critical
Figure 7–4. Optimal feeding position for pro-
moting oral motor skills, safe swallowing and
functional self-feeding abilities. (Illustration by
Kjell Reigstad.)

to understand the implications of various
genetic, neurologic, pulmonary, cardiac,
and gastrointestinal disorders that these
often critically ill infants experience. Prog-
noses for short- and long-term oral feeding
are closely related to the physical and physi-
ologic status. It is as important for the feed-
ing specialist to determine that an infant
is not ready for oral feeding as it is to give
guidance for ways to maximize oral feed-
ing skills and safety in those infants who are
stable and ready to feed orally.
Incidence and Prevalence
of Preterm Births
Current preterm birth rates in the United
States are under 10% (Martin, Hamilton,
Osterman, Driscoll, Drake, 2018). Infants
classified as very low birth weight (VLBW)
made up 1.5% of preterm infants with
birth weight 1500 g. Extremely low birth
weight is 1000 g. Micropreemies are those
with birth weight less than 600 g. Survival
rate has increased with advances in tech-
nology that have not only increased sur-
vival, but have also improved outcomes.
Although atypical per limits of viability, a
case report of an infant born at 21 weeks’
4 days’ gestation showed unimpaired neu-
rodevelopmental outcome at 2 years of age
(Ahmad, Frey, Fierro, Kenton, Placencia,
2017). This infant may be the most prema-
ture known survivor to date. Twenty-year
trends have been reviewed from Neonatal
Research Network Centers (1993–2012)
(Stoll et al., 2015). Data were derived from a
prospective registry of nearly 35,000 infants
born between 22 and 28 weeks’ gestational
age (GA) and with birth weights rang-
ing from 401 to 1500 g. Major morbidities
reported for infants surviving more than
12 hr included severe necrotizing enteroco-
litis, infection, bronchopulmonary dyspla-
sia (BPD), severe intracranial hemorrhage,
cystic periventricular leukomalacia, and/or
severe retinopathy of prematurity. In sum-
mary, changes in maternal and infant care
practices and modest reductions in several
morbidities were observed, although BPD
increased. Survival increased most mark-
edly for infants born at 23 and 24 weeks.
Survival without major morbidity increased
for infants born 25 to 28 weeks’ GA. Authors
suggest that these findings may be valuable
in family counseling and development of
novel interventions.
International variations in gestational
age (GA) distribution of births are reported
acrosshigh-incomecountries(Delnordetal.,
2017) in 27 European countries, United
States, Canada, and Japan. They found that
rates varied from 5.7 to 15.7 per 1,000 total
births and 4.0 to 11.9 per 1,000 live births.
The largest variability was noted with regis-
tration related to percentage of births at 22
to 23 weeks’ gestation (from 1%–23% of very
preterm births) and stillbirths (between 6%
and 40% of very preterm births). Recom-
mendations included exclusion of births at
22 to 23 weeks’ gestation and terminations
of pregnancy. Even so, large rate variations
persisted, with low- and middle-income
countries having the highest rates of pre-
term births. Findings reported by Delnord
et al. (2017) and Vogel et al. (2018) provide
support to the need for continued global
surveillance of preterm births regardless of
the country’s income level.
A rise in early term births has been
documented in the United States from 1989
to the mid-2000s, followed by a decline in
recent years (Buckles Guldi, 2017). The
recent decline in early term births has been
driven by changes in medical practice advo-
cated by the American College of Obstetri-
cians and Gynecologists, programs such as
the March of Dimes’ “Worth the Wait” cam-
paign, and by Medicaid policy. Efforts to

reduce early term elective deliveries appear
effective. Early term inductions result in
lower birth weights and increase the risks of
precipitous labor, birth injury, and required
ventilation. Buckles and Guldi suggest that
reductions in early term inductions can
explain about one-third of the overall in-
creaseinbirthweightsbetween2010and2013
for births at 37 weeks’ gestation and above.
Given the increases in survival and
improved outcomes, a focus on evaluating
infants in the NICU has become widespread.
In addition to knowledge about etiologies
and sequelae associated with prematurity,
clinicians need to have a fund of knowledge
about embryology and genetics. Premature
infants have immature respiratory function,
postural tone, and structural alignment. As
reviewed by Carroll and Agarwal (2010),
although prenatal respiratory control must
be “ready to function” at birth, it remains
immature in the term neonate and more
so in infants born prematurely. Maturation
of respiratory function may take weeks or
months in the term infant and longer in the
preterm infant.
General Principles for
Facilitation of Oral Feeding
Potential for Infants in the NICU
Individualized care in the context of the
family is advocated and includes, but is not
limited to, the following:
n Maintenance of physiologic stability
with appreciation for individual
strengths and needs of the infant with
primary caregivers involved. Interpreta-
tion of communication of each infant
with respect and affirmation of family
desires is fundamental.
n Goals must be experience driven, with
appreciation and understanding of
sequences of development as underpin-
nings for nipple feeding (breast and/
or bottle). Feeding goals cannot be
protocol or template based, or volume
driven without consideration of the
individual infant.
n All professionals need to support
breastfeeding or bottle-feeding of breast
milk, whenever possible, for mothers
and infants. Bottle-feeding can be
provided based on desires stated by the
family and in consultation with NICU
professionals.
Caregiver Involvement
n Caregiver involvement is encouraged by
all professionals, with caregivers present
as much as possible, depending on
family circumstances, jobs, and infant’s
length of stay, to name a few variables.
They are integral partners with other
members of the team. They are primary
decision-makers for their infant with
input from multiple team members in
the NICU. Infant and mother, who is
usually the primary feeder, should be
cared for simultaneously.
n Breastfeeding is the optimal goal for
mothers and their infants. Lactation
consultants and feeding specialists can
assist in this process.
n When parents want their infant to be
bottle-fed, that decision is respected.
Feeding specialists can assist in this
process.
n Parents should be primary feeders
with nurses and feeding specialists
providing demonstration and guidance
throughout the NICU stay.
Environmental Protection
n A primary goal for infants is
nonstressful responses to all proce
dures (to whatever degree possible).

Professionals work to minimize adverse
responses to environmental stimuli
with a goal of pleasurable responses to
all stimuli.
n Professionals provide oral and all other
experiences with each infant, not to the
infant.
n All interventions/feedings should
attend to diminishing adverse responses
from the infant and increasing the
opportunity for interactions with
mother and other primary caregivers.
n Oral experiences that can be facilitated
well before oral feeding readiness is
noted include, but are not limited to,
smells and tastes of breastmilk as well
as nonnutritive sucking (NNS) on a
pacifier or a little finger (reminder to
parents and clinicians: NNS is carried
out with a sucking rate of two sucks per
second, which is twice the rate of nutri-
tive sucking (NS) via bottle and breast
at one suck per second).
Neuroprotection of the
Developing Brain
Neuroprotection and neuroplasticity are two
important and related concepts that have
emerged as being critical to brain develop-
ment and brain healing. Neuroprotection
refers to strategies that prevent neuronal cell
death and enable the brain to heal through
the development of new connections
or pathways (Altimier Phillips, 2013;
McGrath, Cone, Samra, 2011). Neural
protective strategies refer to experiences or
exposures that support brain development
and healing. In contrast, neuroplasticity
refers to the ability of the brain to make
short- or long-term modifications in synap-
tic neuronal connections by incoming stim-
uli associated with activity and experience
(Altimier Phillips, 2013; Pickler et al.,
2010). Currently, seven neural protective
core measures for care are considered essen-
tial to the heathy growth and development
in the preterm infant and family: (a) healing
environment, (b) partnering with families,
(c) position and handling, (d) safeguarding
sleep, (e) minimizing stress and pain, (f)
protecting skin, and (g) optimizing nutri-
tion (Altimier Phillips, 2013; Coughlin,
Gibbins, Hoath, 2009). In addition to
adhering to these core practice tenets, rela-
tive to feeding, these authors (Altimier
Phillips, 2013; Coughlin, Gibbins, Hoath,
2009) also emphasize the following:
n Provision of individualized caregiving
is carried out primarily through
the mother’s regulatory influence,
which is essential for optimal brain
development.
n Caregiving around feeding is focused
on the ultimate goal related directly
to the feeding experience, not volume
consumed.
n Feeding is seen as a neurodevelop-
mental progression with experiences
in the NICU building a foundation for
further learning/development around
eating. Mother or a designee is seen as
the primary provider of sustenance and
nurturing with the infant.
n Breastfeeding is recommended when-
ever possible per caregivers’ desires.
n Bottle-feeding facilitation is encouraged
only when parents/caregivers express
this desire. Parents are always recog-
nized and affirmed as primary feeders,
with a focus on supporting their under-
standing of the infant’s communicative
behaviors.
Late-Preterm Infants
Late preterm is usually defined as birth at
34 0/7 to 36 6/7 weeks’ gestation. Infants

in this gestational age range at delivery are
part of the fastest-growing group that had
often been overlooked in the past as they
were presumed to be essentially “normal.”
However, these infants are known to be
at higher risk of mortality and morbidity
than term newborns (e.g., Hellmeyer et al.,
2012; Kalyoncu, Aygun, Cetinoglu, Kucu-
koduk, 2010). Some examples follow from a
study of 252 late preterm newborn infants
in a tertiary care unit in Turkey (Kalyoncu
et al., 2010): Compared to term infants, late
preterm infants were 11 times more likely
to develop respiratory distress, 14 times
more likely to have feeding problems, 11
times more likely to have hypoglycemia,
3 times more likely to be readmitted, and
2.5 times more likely to be rehospitalized.
Out of 893 late preterm infants reported
in a study from Germany, 59.1% required
intensive neonatal care (Hellmeyer et al.,
2012). In that group, those infants small
for gestational age had a significantly lower
rate of respiratory disorders but were more
often affected by feeding difficulties. Late-
preterm infants in a kangaroo mother care
unit in South Africa demonstrated subtle
breastfeeding difficulties, which high-
lighted the need for breastfeeding support
to mother and infant (Pike, Kritzinger,
Kruger, 2017). Similar findings were
reported by Dosani and public health nurs-
ing colleagues (2017) in Canada. These
authors stressed the importance that public
health nurses receive proper training on safe
and effective breastfeeding of late preterm
infants, so that they can provide anticipa-
tory guidance about possible challenges and
strategies to resolve breastfeeding problems.
Lactation consultants (LCs) are key profes-
sionals to provide guidance for facilitation
of breastfeeding with mothers and infants
(Chamblin, 2009). LCs and SLPs function
as a team for comprehensive coordinated
evaluations in many instances.
Changes in Recent Years:
Initiating Oral Feeding
With Infants on
Respiratory Support
Pre-High-Technology Respiratory
Support Mechanisms
Guidelines have focused on the need for
infants to be off ventilator support for
consideration of oral feeding readiness.
Initial prefeeding respiratory rates (RRs)
when a full-term infant is awake, alert, and
calm are expected to be within a range of
30–60 breaths per minute (BPM) (Crane,
1986; Gould, 1991). Individual variability
and underlying physiologic stability make
definitive guidelines difficult. However,
respiratory rate that increases more than a
few BPM from resting rate may be a sign
that feeding should be stopped. The work
of feeding always has to be put on top of
the work of breathing. Basic guidelines
related to respiratory rate have been and
continue to be helpful to determine whether
a preterm or term infant, who is not sick,
can be given a trial oral feeding during
an initial bedside examination (Wolf
Glass, 1992):
n Oral feeding should probably be
postponed if resting respiratory rate
(RR) is 60 breaths per minute (BPM)
prior to feeding (or more than about 10
BPM above the resting RR).
n Feeding should probably be terminated
if RR goes above 80 BPM during oral
feeding.
n When RR increases during feeding,
infants should be monitored after
feeding to determine how long it
takes for return to baseline values,
given a prolonged recovery is a sign
of stress on the infant (Wolf Glass,
1992).

Despite the presence of both swallow-
ing (11–12 weeks) and sucking (18–24
weeks) behaviors in utero, suck–swallow
and breathing are not expected to be coordi-
nated well enough for successful oral feed-
ing to meet nutritional needs until about 33
to 34 weeks’ gestational age in most preterm
infants. Some healthy preterm infants may
demonstrate readiness for oral feeding by
32 weeks and, in some instances, even ear-
lier. Once infants can tolerate enteral feed-
ings, they usually get nutritional needs met
via orogastric (OG) or nasogastric (NG)
tube feedings. Until premature infants dem-
onstrate readiness for essentially all feedings
orally, they continue with OG or NG tube
feeds for assurance of meeting nutritional
needs. “Normal” premature infants of 34 to
36 weeks’ gestation (now classified as late
preterm infants) may demonstrate skills
that show positive prognosis for becoming
full oral feeders, but in fact they may not
consistently take oral feedings efficiently
until closer to 37 weeks’ gestation. In some
instances, total oral feeding may not be
achieved until even later. Quality of oral
feeding is more important as a foundation
for successful feeding over time than exces-
sive focus on increasing volume.
Skin-to-Skin Contact
Skin-to-skincontact(SSC)betweenamother
and her newborn infant (Conde-Agudelo
Diaz-Rossello, 2016) was often referred
to as Kangaroo Mother Care (KMC). SSC
is the more accurate term that also sup-
ports contact between father and infant.
SSC in low birth weight (LBW) infants is
supported, particularly in resource-limited
settings. SSC is also supported for mothers
and their healthy newborn infants (Moore,
Bergman, Anderson, Medley, 2016).
Oral Feeding With Nasal
Continuous Positive Airway
Pressure and High-Flow
Nasal Cannula
Use of nasal CPAP has been shown to
reduce length of oxygen dependence and
hospital stay in premature infants born at
VLBW (1.5 kg) and extremely low birth
weight (1 kg) who often have long lengths
of stay in the NICU, which is a heavy health-
care resource burden (Davis Henderson-
Smart, 2003). Global drawbacks with CPAP
are reported to include, but may not be
limited to, need for intensive nursing, nasal
breakdown, and poor tolerance by some
patients (Bonner Mainous, 2008; McCo-
skey, 2008).
Use of heated humidified HFNC is
increasing throughout the world as a nonin-
vasive respiratory support for weaning from
CPAP in the NICU setting (Manley et al.,
2013; Ojha, Gridley, Darling, 2013; Sasi,
Malhotra, 2015). HFNC is flow based,
whereas CPAP is a pressure-based sup-
port. Benefits of HFNC include, but may
not be limited to, ease of use, reduction in
nasal trauma, lower pain scores, improved
nursing care, and improved infant–parent
bonding (e.g., Hough, Shearman, Jardine,
Davies, 2012; Osman, Elsharkawy, Abdel-
Hady, 2015). A randomized control trial in
Ireland revealed that preterm infants treated
with HFNC did not achieve full oral feed-
ing more quickly than infants treated with
NCPAP (Glackin, O’Sullivan, George, Sem-
berova, Miletin, 2017).
Potential for introduction of oral feeding
while preterm infants are on HFNC is shown
to be positive in neonates who were deemed
developmentally and medically appropriate
by neonatologists and nurses to being oral
feeding. Leder, Siner, Bizzaro, McGinley,
and Lefton-Greif (2016) found that all neo-

nates meeting those criteria were successful
with initiation of oral feedings. This group
suggests that patient-specific determinants
of swallowing and feeding readiness are pri-
mary. A very low flow rate is likely used for
HFNC in infants ≥32 weeks’ gestation, who
are stable and demonstrate the prerequisite
neurodevelopmental maturation for feed-
ing. It is important to consider the under-
lying medical conditions, reasons for the
HFNC, and flow rate when assessing readi-
ness for oral feeding. HFNC does provide
better access to the infant’s face, which may
aid in improvement of nursing, feeding, and
bonding (Ciuffini et al., 2014). Thus far,
the range of rates compatible with feeding
readiness and long-term feeding outcomes
for infants on HFNC is not known (Leder
et al., 2016; Sasi Malhotra, 2015). Thus,
feeding specialists communicate with pro-
fessionals and parents in the NICU on the
basis of extensive knowledge of underlying
diagnoses and more importantly the infant’s
status in all areas.
Roles of Feeding Specialists
and Others When Oral
Feeding Is Not Yet Feasible
Feeding specialists may be consulted for
consideration of oral feeding for infants
once they are medically and surgically sta-
ble. Specialists also may be consulted for
infants who clearly are not ready to begin
oral feeding. With adherence to neural pro-
tective core measures, these infants may
benefit from nonnutritive stimulation to
provide opportunities for sucking on a paci-
fier or a caregiver’s little finger, along with
partial nipple feeds, as tolerated (Chap-
ter 9). These activities can help to prepare
for advancing oral feeding as they assist in
normalizing all aspects of development.
In some situations, professionals can help
prepare families for the potential of long-
term nonoral feeding needs. Early identi-
fication of neonates at risk for developing
feeding problems in infancy is important
for prevention of severe problems in many
instances (e.g., Hawdon, Beauregard, Slat-
tery, Kennedy, 2000).
Bedside Evaluation
Procedures in NICU and
Other Inpatient Units
After a thorough reading of the medical
chart, a clinician begins a bedside evalua-
tion upon first glimpse of the infant. These
basic guidelines apply to young infants who
are inpatients in other units, as well as those
in the NICU. Not all infants in a NICU are
preterm infants. Important observations of
the infant at rest are made to note:
n general appearance (well or sick
appearing);
n state, posture, and position;
n responses to stimuli (e.g., touch, light);
n respiratory status that includes rate and
effort of breathing;
n heart rate; and
n oral peripheral mechanism.
Any asymmetry of the extremities, trunk,
and especially the face is noted. Infants with
increased tone and hypersensitivity to stim-
uli tend to have uncoordinated sucking. In
contrast, infants with decreased tone and
lack of sensory arousal frequently have poor
initiation of sucking and suck movements
are weak. Open-mouth posture that can
indicate mouth breathing raises the possi-
bility of low tone or upper airway obstruc-
tion, given that infants are described as
predominantly obligate nose breathers in

order to take nipple feeding at breast or by
bottle. Premature infants may be unable to
integrate sensory input from the environ-
ment. The examiner must be alert to signals
that indicate stress or lack of integration.
These signals may include dusky skin color,
irregular breathing patterns, overall agitated
state, splaying of fingers, and reduced level
of alertness.
Oral sensorimotor patterns of preterm
infants, as well as other physical and physi-
ologic attributes, are different from term
infants (Table 7–8). Infants with poor suck-
ing abilities have been found to require
significantly longer feeding times (Casaer,
Daniels, Devlieger, DeCock, Eggermont,
1982). Gavage feeding or prolonged use of
endotracheal tubes may delay or alter the
development of function for oral feeding.
In some infants, oral function can become
disorganized with decreased sucking abili-
ties. Coordinated nonnutritive sucking and
swallowing are necessary but not sufficient
for the development of an adequate suck/
swallow/breathe pattern for nutritive pur-
poses in infants (Daniels, Devlieger, Casaer,
Callens, Eggermont, 1986).
If there are no contraindications for
continuation of the examination, the infant
can be placed in an appropriate position for
feeding. The infant may attain and main-
tain a calm, alert state if swaddled and held
semi-upright in a neutral posture or in side-
lying position that keeps the trunk, neck,
and head in a neutral straight line. Respira-
tory rate should stay in the range of 40 to
60 breaths per minute (BPM). Tachypnea
(rate 60 breaths per minute in infants) may
be due to pulmonary, cardiac infectious or
metabolic conditions in this population.
Understanding the reason for the tachy-
pnea is essential. When infants present with
tachypnea secondary to underlying cardiac
conditions, feeding readiness is contingent
Table 7–8. Comparison of Physical and Physiologic Attributes for Feeding
Readiness in Preterm and Term Infants
Attribute Preterm Infants Term Infants
Position/posture Extensor Flexor
Trunk, shoulder, and neck stability Poor Good
Anatomic set for sucking Not favorable Favorable
Sucking strength Weak Strong
Lip seal Inadequate Adequate
Cheek stability Unstable Good
Jaw stability for sequential sucking Insufficient Sufficient
Hunger signals Inadequate Adequate
Neurologic status Disorganized, irritable Organized
Suck/swallow/breathe pattern Dysrhythmic Rhythmic
Oral reflexes Incomplete Intact
Source: Adapted from Morris, S. E., Klein, M. D. (2000). Pre-feeding Skills: A Comprehen-
sive Resource for Feeding Development. Tucson, AZ: Therapy Skill Builders.

upon clearance by their cardiologists and
cardiovascular surgeons (Ehrmann et al.,
2018). Check for rooting responses, which
should be seen when infants are hungry as
they search for the breast or other nipple. If
rooting is not spontaneous, consider strok-
ing rhythmically from ear to mouth in a
firm but gentle way at a rate of one stroke
per second. This is the rate of nutritive suck-
ing. The next step is evaluation of nonnutri-
tive sucking (NNS) as a necessary, but not
sufficient, skill for an infant to be successful
at nipple-feeding by breast or bottle.
Nonnutritive Sucking (NNS)
NNS may be observed in multiple ways.
Some infants may be sucking on their
tongue, fingers, or a pacifier when the feed-
ing specialist approaches the bedside, or
in case of outpatients, when the infant is
brought into a clinic room. Some infants
never take pacifiers; some do not hold
pacifiers in their mouths. It is important
for those infants who do use pacifiers that
they demonstrate “stripping” action of the
tongue in ways that are as similar as possible
to the patterns that are needed for nutritive
sucking, Short flat or NUK-type pacifiers
typically are not appropriate to promote
the sucking patterns needed regardless of
whether direct breastfeeding is the goal or
bottle-feeding is being considered either in
total or as supplemental feedings. Clinicians
are urged to evaluate the neurophysiologic
patterns of sucking, to describe those prin-
ciples to parents, and to make recommenda-
tions appropriate for each infant. The task is
not straightforward given the considerable
variability in characteristics of pacifier com-
pression, pull stiffness, and nipple shape
that in turn yield different NNS dynam-
ics (Zimmerman, Forlano, Gouldstone,
2017). Zimmerman and colleagues carried
out a mechanical test of seven commonly
used pacifiers, which revealed that vari-
abilities are found across manufacturers and
distributors as well as within brands. Find-
ings do not provide information that can be
carried over directly to individual infants.
They emphasize the need for further inves-
tigation into pacifier properties and sucking
patterns in young infants.
During evaluation of NNS, it is not pos-
sible to determine the tongue action just by
observation of an infant sucking on a paci-
fier. The examiner can ease a gloved little
finger slowly into the infant’s mouth to
mid-tongue. Infants with strong, rhythmic
sucks may “pull” the examiner’s finger into
the mouth. The finger should be placed no
further posteriorly than the upper border of
the soft palate so as not to elicit a gag reflex
(McBride Danner, 1987). This process is
accomplished with the infant held so that
the head is in neutral position. NNS or
mouthing on the examiner’s little finger
for 1 min is measured in sucks per second
(Case-Smith, Cooper, Scala, 1989). The
rate of NNS should be two sucks per second.
If no sucking action is noted, try stroking
the tongue or the hard palate from mid to
front at the rate of one stroke per second
(nutritive sucking rate) four to six times,
then stop and hold the finger in the infant’s
mouth. Repeat after several seconds up to
10 to 12 times. Slow removal and replace-
ment of the finger on the tongue at least
twice allows for observation of initiation
of movement, cupping of the tongue, and
extension and retraction of the tongue. Pac-
ifier use has been shown to accelerate tran-
sition to full breastfeeding and to improve
sucking skills in preterm infants (e.g., Kaya
Aytekin, 2017). Pacifier use started at
birth has been shown not to have a nega-
tive effect on the prevalence or duration of
exclusive and partial breastfeeding up to
4 months of age (e.g., Jaafar, Ho, Jahanfar,
Angolkar, 2016).

The “normal” infant closes the mouth
and immediately initiates a sucking action.
The tongue of an infant with hypotonia
will feel soft and can be moved easily by
the examiner. In contrast, the tongue of an
infant with hypertonia may be firm and con-
tracted, likely retracted. An excessive bite
is present if the jaws clamp firmly against
the finger and sucking is not initiated. NNS
can be assessed immediately before an oral
feeding trial or a nonoral tube feeding, as
well as during a tube feeding. Breathing
patterns are observed for changes in rate
and rhythm. The presence of any respira-
tory noises during NNS and swallow are
also noted. In most instances, infants with
signs of respiratory distress require in-depth
airway examination before evaluation of
nutritive sucking is undertaken (Chapter 4).
There may be some exceptions when clini-
cians raise questions about airway status,
and they will proceed to a limited oral feed-
ing evaluation that can provide additional
information useful to the otolaryngologist
prior to the airway examination. Deci-
sions regarding oral feeding trials take into
account multiple factors.
An adequate NNS pattern may not nec-
essarily lead to adequate nutritive sucking
for successful oral feeding. Nonetheless,
some benefits of NNS have been demon-
strated to facilitate a more rapid transition
from gavage to full oral feeding and earlier
discharge from NICU to home, although
findings vary across studies (e.g., Arved-
son, Clark, Lazarus, Schooling, Frymark,
2010; Bingham, Ashikaga, Abbasi, 2010),
for increasing transcutaneous oxygen ten-
sion in infants between 32 and 35 weeks’
postconceptional age (Paludetto, Robertson,
Hack, Shivpuri, Martin, 1984), and for
increasing weight gain (Field et al., 1982).
If the infant does not demonstrate a
functional nonnutritive suck, the clinician
should make recommendations for NNS
that may help prepare the infant for oral
feeding. If the infant demonstrates a func-
tional NNS and has a stable airway, proceed
with an oral feeding evaluation.
Oral Feeding Evaluation:
Three Groups of Infants
This section presents principles of bedside/
clinical oral feeding evaluations of three
groups of children: (a) infants in the NICU,
(b) infants beyond the NICU with a feed-
ing specialist and lactation consultant, and
(c) infants who are total bottle-feeders.
Infants in NICU
Nutritive Sucking and Swallowing
The evaluation is most likely targeted toward
bottle-feeding of breast milk or formula,
unless the mother is in the NICU and pre-
pared to breastfeed with anticipated guid-
ance from a lactation consultant. If the infant
has an OG or NG tube, it may be removed
for the feeding evaluation if the infant has
shown readiness for possible total oral feed-
ing. If, however, history and physical find-
ings suggest that the infant is not likely to
take much liquid orally, it is better to leave
the tube in place. NG tubes may have effects
in VLBW infants that impact airway stabil-
ity (e.g., decreased nasal airflow, increased
airway resistance, and abnormal airway
distribution). An intermittent NG tube may
create other problems: insertion stimulates
the larynx and may cause laryngospasm.
Apnea and bradycardia are more likely,
and pharyngeal and esophageal trauma are
possible (Symington, Ballantyne, Pinelli,
Stevens, 1995). An NG tube in place before
feeding can affect the start of the oral feeds

with lower tidal volume and lower minute
ventilation. An NG tube in place during
feeding may affect the feeding by decreasing
tidal volume, minute ventilation, pulse rate,
oxygen saturation, force of sucking, and vol-
ume consumed (e.g., Daga, Lunkad, Daga,
Aluja, 1999; Greenspan, Wolfson, Holt,
Shaffer, 1990; Shiao, Youngblut, Anderson,
DiFiore, Martin, 1995).
The evaluation of oral feeding should
be carried out with an infant feeding for at
least 15 to 20 min, which is sufficient for a
premature infant to take the full feeding if
he or she is efficient. In that time a clinician
should be able to determine whether the
infant becomes disorganized or fatigued as
the feeding continues. These observations
are important for potential recommenda-
tions about an oral feeding plan. The effi-
cient feeder is expected to suck at a rate of 1
suck per second, in bursts of 10 to 30 suck/
swallow sequences, followed by a pause of
1 or 2 s as the infant takes a breath and swal-
lows an additional time or two. The pattern
is repeated for the duration of the feeding.
Sucking bursts are likely to get shorter near
the end of the feeding. The feeding special-
ist may alter nipples and containers if the
infant does not appear to be calm, coordi-
nated, or efficient with the first system. It
must be remembered that there is no per-
fect nipple. It seems that each year there
are more bottle/nipple systems coming
available, each one marketed as the “best”
in varied dimensions. Flow rates are shown
to vary within and across brands of nipples
(e.g., Pados, Park, Thoyre, Estrem, Nix,
2015). Viscosity can be changed as a means
of altering flow rate. When flow rate is too
fast, the infant will have to work to slow it
down relative to airway protection. Alter-
natively with thicker liquids, the infant
will need to work harder to extract the
fluid from the nipple. Most infant assess-
ments consist of descriptive observations,
which are compared with normal develop-
ment. Deviations from “normal” are noted,
although data-based evidence for “normal”
is lacking.
Breastfeeding Facilitation
Lactation consultants, usually nurses with
extensive training and credentials or other
professionals who have obtained the certi-
fication through the International Board of
Lactation Consultant Examiners (IBLCE).
Breastfeeding is encouraged for all infants
and mothers whenever it is possible with
a worldwide reach. Multiple factors likely
contribute to decision-making. Resources
for parents and clinicians include, but are
not limited to, books by Genna (2016); War-
ren and Bond (2014); Zaichkin (2010); and
Zaichkin, Weiner, and Loren (2016).
Decisions about management recom-
mendations (Chapter 9) have to take into
account multiple factors related to the
infant’s underlying physical and physiologic
status, as well as the performance during the
feeding evaluation and factors unique to the
specific infant–mother dyad. Neonates (first
28 days following birth), particularly VLBW
infants, with disorganized or dysfunctional
feeding are at high risk for longer-term
feeding problems that may include difficul-
ties in making the transition to solid food
at 6 months corrected age and for tolerat-
ing lumpy food by 12 months corrected age
(Hawdon et al., 2000).
Evaluation of Infants
Beyond NICU
Preterm and term infants differ in a num-
ber of attributes that have an impact on oral
feeding (see Table 7–8). By the time infants
reach 39 to 40 weeks’ gestation or term,
they demonstrate physiologic flexion of the

limbs that is one of the attributes contrib-
uting to successful oral feeding. Rhythmic
suck/swallow/breathe coordination with
appropriate rate of one suck per second is
another necessary attribute.
Prefeeding Observation
The same principles used in the NICU hold
for observation of infants prior to feeding.
Professionals should approach infants with
intention to watch and listen before han-
dling. The infant is first observed when
awake and not being stimulated so that
breathing patterns can be observed along
with the level of spontaneous activity.
A complete assessment of oral struc-
tures and function is made before any intro-
duction of liquid. Lip action is important
for latching to the breast and getting a seal
for both breast- and bottle-feeding. Visual
inspection of the structures in the mouth
(e.g., tongue, palate, and buccal muscu-
lature) permits identification of any oral
structural abnormality that could interfere
with sucking and swallowing. It is impos-
sible to observe the pharynx.
The presence of rooting responses and
the quality of tongue action provide evi-
dence for feeding/swallowing specialists
to determine readiness to suck and swal-
low liquid. A common rooting response
of normal infants is a head turn toward
the side being stroked. Other infants may
make side-to-side head movements while
turning toward the side where stroking
occurred. Infants will search by moving the
lips and head to try to take the stimulus into
the mouth.
Laryngeal function for airway protec-
tion is inferred by perceptual interpretation
of voice quality. Voice quality may change
when the child’s position for feeding is
changed. The clearest voice quality is usually
noted when the infant is in the best position
for feeding, whether nearly upright, sidely-
ing, or semiprone. A “gurgly” voice quality
may indicate secretions in the pharyngeal
recesses. However, normal phonation is
only an indication of laryngeal function for
phonation, but with no evidence for air-
way protection during swallowing. These
observations may hint at possible pharyn-
geal function, but they are not definitive.
At most, the “gurgly” voice is one of many
signs that contribute to decision-making
about whether or not to move ahead with
oral feeding.
Cleft Palate With or
Without Cleft Lip
A cleft of the palate, with or without cleft lip,
may be observed (Chapter 12). A submu-
cous cleft of the soft palate, characterized
by notching at the junction of the hard and
soft palate with a zona pellucida (indicat-
ing submucosal diastasis of palatal muscu-
lature) and a bifid uvula (Figure 7–5) , may
or may not be symptomatic in relation to
feeding function.
Figure 7–5. Cleft of soft palate with bifid uvula.
(From https://elementsofmorphology.nih.gov/
index.cgi?tid=30b9e9da9758d9d7)

Intubation Groove
Shape and height of the hard palate are also
noted. Children who were intubated orally
for as few as 2 weeks during the newborn
period may develop a persistent narrow
groove in the midline of the hard palate.
This complication has been reported up to
87.5% of orally intubated infants (Erenberg
Nowak, 1984). Enomoto and colleagues
found palatal groove in 14 of 37 infants with
low birth weight who then were delayed in
advancing oral feeding (Enomoto et al.,
2017). Palatal stabilizing devices (PSDs)
are shown to reduce spontaneous acciden-
tal extubations and may provide preventive
measures. There were no other complica-
tions of intubation (Fadavi, Punwani,
Vidyasagar, 2000; Testa, Fadavi, Koerber,
Punwani, Bhat, 2012). Palatal groove for-
mation is also reported in infants who were
fed for prolonged periods with OG tubes
(Arens Reichman, 1992). The palatal
architecture is disrupted, and these children
exhibit a high incidence of enamel defects
in primary dentition. Preventive measures
include acrylic palatal appliances or pref-
erential use of NG tubes. As children get
older, food may get lodged in the groove.
A high arched palate has no proven negative
effects on oral–motor function.
Ankyloglossia (Tongue Tie)
and/or Upper Lip Tie
There is wide variation in prevalence of
ankyloglossia worldwide. Estimates vary
from 4% to 11% (O’Shea et al., 2017). Over
the past 10 years the frequency of new-
borns diagnosed and treated for tongue tie
has increased globally. For example, frenot-
omy rates in Australia increased by 420%
between 2006 and 2016 (Kapoor, Douglas,
Hill, Walsh, Tennant, 2018). Despite these
trends, data supporting improvements in
breastfeeding following frenotomy are lim-
ited. Walsh and Tunkel (2017) reviewed the
potential difficulties in attaining objective
data supporting or refuting the impact of
frenotomy on breastfeeding. Perceptions
appear to be broad based that these proce-
dures are low risk, may have strong benefits,
and are supported by family preferences and
social media. Despite the assumption of low
risk, airway obstruction has been reported
in two patients with Pierre Robin pheno-
types who underwent frenotomy (Genther,
Skinner, Bailey, Capone, Byrne, 2015).
The support of successful breastfeeding
has led to increased assessment of tongue
movements of infants during problem-
atic breastfeeding. Ingram and colleagues
reported that the presence of tongue-tie in
an infant may lead to breastfeeding diffi-
culties (Ingram et al., 2015). These authors
developed a four-item Bristol Tongue
Assessment Tool (BTAT) with good internal
reliability (no information about validity).
This brief tool correlates well with the Hazel-
baker Assessment Tool for lingual frenulum
(ATFLL), which is used by Hazelbaker in
her own detailed breastfeeding assessments
(Hazelbaker, 2010). The four items in the
BTAT for assessment are tongue tip appear-
ance, attachment of frenulum to lower gum
ridge, lift of tongue with mouth wide(ideally
viewedwheninfantiscrying),andprotrusion
of tongue. Each item is rated 0, 1, or 2, with
2 essentially normal. Individual scores are
summed giving a range from 0 to 8. Authors
state that scores of 0 to 3 indicate more severe
reduction of tongue function (Ingram et al.,
2015). This assessment is carried out before
oral feeding is observed. Management guide-
lines are found in Chapter 9.
Upper/maxillary lip tie (superior labial,
maxillary labial frenulum) is less common
than the restricted lingual frenulum. When
breastfeeding difficulties are reported, it
is particularly important to inspect the

attachment of the upper lip to the maxillary
gingival tissue. There is no muscle contained
within this tissue. Kotlow (2013) provides
in-depth discussion of the diagnosis and
classifications of lip-tie with particular
attention to the effect on an infant’s latch
to the mother’s beast. Lip ties that interfere
with the infant’s ability to flare out the upper
lip must be considered as a possible impedi-
ment for successful breastfeeding. Parents
often find it useful to hear the description
of lip configuration appearing like “fish
lips,” since both upper and lower lips need
to flare out for successful latching at breast
and bottle nipple. Upper lip-tie release led
to improved breastfeeding in all 14 infants
treated by Pransky, Lago, and Hong, 2015.
Observation of Infant
Bottlefeeding
In the inpatient setting, the first oral feeding
observation is performed after the infant’s
cardiac and respiratory status are stable,
bowel sounds are adequate, and feedings
can be done with minimal respiratory dis-
tress . Three major goals for all feeding/
swallowing examinations and management
recommendations are (a) safe feeding with
minimal risk for aspiration, (b) functional
feeding with sufficient nutrition and caloric
intake to ensure weight gain within a rea-
sonable length of time at each feed, and
(c) pleasurable feeding. Unless pleasurable
nonstressful feeding occurs regularly and
efficiently, the infant is in danger of under-
nutrition or malnutrition (Ross Philbin,
2011). To reiterate, adequate weight gain is
critical in the first months of life.
Observation of the infant feeding for at
least 15 to 20 min is desirable. Some infants
appear to have adequate coordination for
the first few minutes but cannot sustain the
necessary rhythmic suck/swallow/breathe
patterns long enough to take sufficient
quantity to meet nutrition needs. A rhyth-
mic pattern of nutritive sucking is expected
to occur at a rate of one suck per second.
The sucking sequence consists of “bursts” of
suck/swallow sequences followed by a brief
pause of 1 to 2 s. On average, up to 25 to 26
sucks may characterize the burst sequence
in term infants who are 1 and 2 days of age
(Medoff-Cooper, Bilker, Kaplan, 2010).
A typical healthy infant appears to be suck-
ing nearly constantly, with frequent swal-
lows and appropriate breaths allowing for a
relatively large volume of liquid consumed
in a short period of time without aspiration
concerns. The most efficient pattern is 1:1
suck:swallow. Suck to swallow sequencing
up to two to three sucks per swallow is con-
sidered functional (Gewolb Vice, 2006).
An infant who sucks five to six or more
times before producing a swallow is work-
ing too hard and likely will tire out before
completing an adequate feeding. This infant
also may be at higher risk for aspiration
with a small amount of liquid getting into
the pharynx with each suck that could result
in laryngeal penetration or even aspiration
before a swallow is produced. The examiner
watches for signs of increased cardiac or
respiratory rate/effort that may include, but
are not limited to, dysrhythmia of cardiac
and respiratory patterns, gagging, spitting,
tongue thrusting, squirming and withdraw-
ing, arching of the back or neck, dribbling
of formula/breast milk, and falling asleep.
Lip closure, tongue action, cheek posture,
and laryngeal movement are all observed.
Infants with neurologic impairments who
demonstrate sucking difficulty are at in-
creased risk for aspiration during nipple
feeding. Suctioning capabilities should be
readily available during the assessment of
some high-risk infants.

Nipple and Viscosity Variables
The types of nipple and viscosity of the liq-
uid have been shown to influence the suck-
ing behavior of neonates and young infants.
Nipple pliability and size of the opening are
both important (Adram, Kemp, Lind,
1958; Dubignon Campbell, 1968). Pados
and colleagues (2015) found considerable
variability in flow rates within and across
brands of nipples for feeding infants who
are hospitalized (Pados, Park, Thoyre, Es-
trem, Nix, 2015) and for nipple used after
hospital discharge (Pados, Park, Thoyre,
Estrem, Nix, 2016). Each year more nip-
ple and bottle systems appear on the mar-
ket, whether in stores or attainable online,
which means specific recommendations are
difficult to make. Brown (1972) found that
infants responded preferentially to a regu-
lar rounded nipple shape compared with
a blunt NUK-type nipple. The NUK-type
nipple is fairly flat and short with an almost
“hook” so that the infant cannot “strip” that
type of nipple. It is not unusual for leakage
out of the corners of the mouth since liquid
is released farther forward on the tongue
than with a “standard”-type nipple. Thus,
feeding/swallowing specialists need to be
aware that infants vary in nipple prefer-
ences, so more than one type may need to
be tried (Mathew, Belan, Thoppil, 1992).
However, there is no perfect nipple—adjust-
ments may be made in position of the
infant, viscosity of the liquid, or some other
factors (Chapter 9). It is also important that
the underlying cause for the sucking diffi-
culty be determined so the clinician has a
basis for assessing the infant with multiple
nipples and containers. Nipple preference
may change over time, especially for those
infants who start out with a small, soft pree-
mie nipple and after a few weeks progress to
a longer, firmer nipple.
Satiation Influences
Satiation also influences sucking. From the
first day of life, the presence of milk in the
stomach seems to be an inhibitor of nutritive
sucking (Bergman 2013; Satinoff Stanley,
1963). Infant feeding cues may be important
for successful feeding at breast and bottle
(Shloim, Vereijken, Blundell, Hethering-
ton, 2017). Shloim and colleagues (2017)
found that significantly more frequent feed-
ing cues were observed at the beginning
than at the end of the feeding indicating
that cue frequency changes with satiation.
Breastfeeding infants showed more engage-
ment and disengagement cues than formula-
fed infants. These cues related to hunger
and satiation continue to be important in
the first few years of life. Further research is
needed (McNally et al., 2016).
The sucking rate also varies with the
concentration of sucrose. Burke (1977)
found that swallowing activity increased
relative to the number of sucks, and sucking
rate slowed as amount and concentration of
sucrose were increased. Infants have been
found to suck at higher rates for formula
with a higher relative viscosity (Kron, Stein,
Goddard, Phoenix, 1967). Thus, assess-
ment at what would be the typical feeding
time as well as the use of various nipples,
containers, and even formulas may influ-
ence the outcome of the evaluation.
Neonatal Feeding Intervals
Evidence is limited regarding feeding inter-
vals during the first month of life. A review
of the literature by Bergman (2013), sug-
gests that a stomach capacity of approxi-
mately 20 ml could determine feeding
frequency at birth. That stomach capacity
would correspond to a feeding interval of
about 1 hr—the gastric emptying time for

human milk. This 1-hr time frame is also
the normal neonatal sleep cycle. Bergman
speculates that larger feeding volumes at
longer volumes may be stressful to infants
and the cause of spitting up, reflux, and
hypoglycemia. Bergman added that these
considerations would be consistent with
evolutionary expectations for human neo-
nates. Further research is needed to deter-
mine best practices for optimizing nutrition
in nonstressful ways for infants as well as
their parents/caregivers.
Examination of Infants on
Nonoral Tube Feeding
Infants on OG or NG tube feedings for
nutrition needs can be examined for initial
oral feeding with an NG tube left in place,
particularly if it is likely that tube feeding
supplements will continue to be needed
at least in the short run. When an infant
is anticipated to reach total oral feeding
within a few days, a tube may be removed
for the feeding evaluation. Total oral feeding
will likely occur following gradual advance
with increased nipple feeding and reduced
tube feeding unless there are extenuating
circumstances (e.g., neurologic, cardiac, or
gastrointestinal problems). Feeding special-
ists may want OG tubes removed when they
carry out an examination since OG tubes
can interfere with oral feeding by preventing
adequate lip closure and tongue movement.
Inadequate lip closure and limited tongue
action may interfere with effective buildup
of intraoral pressure during sucking. OG
tubes are used for infants with small nares
or across ages following surgery or trauma
to the nasal area. Evidence is needed regard-
ing whether OG or NG tubes may affect the
pharyngeal area and in turn what effect that
will have on the coordination of suck–swal-
low patterns. The feeding assessment should
be carried out in as normal conditions as
possible. Some believe that the tube cannot
be replaced immediately after the infant has
taken some liquid because of increased risk
for emesis. The feeding schedule may then
be disrupted if the required volume of liquid
cannot be given to the infant within approx-
imately 30 min. Serial assessments may be
needed over several feeding times, or even
over several days, to monitor change in the
infant, to establish an oral sensorimotor and
feeding plan, and to assist in implementing
recommendations.
An important consideration in decision-
making for oral feeding readiness of a tube-
fed infant is tolerance of bolus tube feedings
that closely mimic oral feeding. Typically
optimal volume bolus feedings over 20 to
30 minutes every 2 to 3 hours mimic normal
hunger and wake-sleep cycles. In contrast
to bolus feeding, slow and continuous tube
feedings are delivered over longer periods
of time and some over 18 to 24 hours. Slow
and continuous feedings are needed when
the infant’s gastrointestinal system can-
not handle larger quantities in short time
periods. Unfortunately, continuous feed-
ings may interfere with hunger and satiety
cycles. When professionals and parents esti-
mate that it may require several months or
even years to attain total oral feeding, a gas-
trostomy tube is preferred over an NG tube.
Other chapters cover discussion of inter-
vention with strategies for transitioning to
different types of feeding tubes (Chapter 5)
and from tube to oral feeding (Chapter 9).
General or Global
Feeding Observations
Once the global physical examination with
oral peripheral examination is completed,
the clinician should observe a primary
caregiver feeding the infant as typically as

possible. Exceptions are made when a pri-
mary caregiver is not available in the NICU
or other inpatient setting. In those situa-
tions, a feeding specialist or nurse may feed
the infant.
Feeding is an interactive process involv-
ing a division of responsibility no matter
what the age is of an infant or child. The
feeder is responsible for what to feed, and
the child is responsible for how much to
eat/drink (e.g., Satter, 2013). The feeder
chooses breast- or bottlefeeding, then helps
the infant to be calm and alert by paying
attention to the infant’s cues. Observations
are made of child and caregiver actions and
interactions. A basic cranial nerve examina-
tion is carried out (Table 7–9). Therapeutic
adjustments may be considered as the feed-
ing session progresses, providing the infant
appears to have a safe swallow (details in
Chapter 9). The clinician may be able to
suggest changes that could include position
and posture, types of nipples, fluid viscosity,
and ways to work through sensory issues.
If there are concerns about risks for aspira-
tion, safety of the airway, or possibilities of
GERD/EERD, the clinician makes recom-
mendations for additional consultations or
testing. If there are concerns related to nutri-
tion status, a dietitian should be consulted.
Cervical Auscultation
Is there a role for feeding specialists to use
cervical auscultation (CA) as they evaluate
children for swallowing and feeding? What
evidence is there for validity and reliability
of CA? Conclusions from a recent system-
atic review were that there is no available
evidence for the validity and reliability of
CA, and CA should not be used as a stand-
alone instrument to diagnose dysphagia in
children (Lagarde, Karmalski, van den
Table 7–9. Observations Correlating With Cranial Nerve (CN) Function During Feeding
Evaluation (Infant and Older Child)
Cranial Nerve Stimulus Normal Response Deficit Response
V Food on tongue Mastication initiated Bolus not formed
Mandible movements
limited or incoordinated
VII Sucking Lips pursed to latch
on to nipple
Lack of lip seal on nipple
Food on lower lip Lip closure Lack of lip movement
Smile Retraction of lips Asymmetry or lack of
retraction
IX, X Food posterior in
mouth
Swallow 2 s Delayed pharyngeal
swallow
Soft palate elevation
and retraction
Nasopharyngeal reflux
XII Food on tongue Tongue shape, point
and protrude
Lack of tongue movement
or incoordination, excessive
thrust, fasciculations

Engel-Hoek, 2016). Multiple variables make
the use of CA problematic—differences in
stethoscopes, lack of measurable swallow
sounds, lack of objective evidence for pro-
cedures, and lack of correlation to swallow-
ing patterns as delineated by instrumental
swallow evaluations (Chapter 8). Hence, the
evidence does not support the use of CA in
clinical feeding evaluations, even though
CA is considered by some as a noninvasive
measure of swallowing, At best, CA may be
useful for screening but not appropriate for
definitive stand-alone diagnosis.
Voice Quality Observations
What can we learn from voice quality obser-
vations? A “gurgly” voice quality raises con-
cern about risk for aspiration with indica-
tions that secretions and/or liquid may
have spilled into the laryngeal vestibule or
lower in the airway. A breathy, weak, husky
to hoarse voice raises concerns for possible
vocal fold paralysis or paresis that may pre-
dispose to aspiration, especially on thin liq-
uids. Awareness of the relationship of these
vocal quality changes to impaired swallow-
ing will alert the clinician to a need for addi-
tional diagnostic studies of the airway and
swallowing function.
Steps in Feeding
Observation of
Older Children
Texture Considerations
Expectations for expansion of textures in
typical children follow a sequence that may
have considerable variation in actual tim-
ing, but the order appears similar from one
child to another. Differences from typical
expectations provide a basis for clinicians
to form impressions that can subsequently
be used to estimate delay or disorder of
oral skills as well as perceived safety for
oral feeding in children with feeding/swal-
lowing disorders. Corrected age is used for
children born prematurely until they reach
24 months chronologic age. Global devel-
opmental levels factor into estimations of
appropriate expectations and are more basic
than age of child.
n Liquid only by nipple first 4 to 6 months
(breast milk and/or formula).
n Strained smooth food by spoon
(6 months) when typical children are
sitting with minimal support and their
gastrointestinal tracts have matured so
that they can tolerate additional types
of food.
n Lumpy foods by 10 to 11 months (avoid
mixed textures, e.g., vegetable soup,
yogurt with fruit bits) increase texture
gradually in small steps to thicker,
grainier, and slightly lumpy, but not
chunky, foods.
n Finger foods that are introduced as easily
dissolvable foods or soft solids may
range from 7 to 8 months to 11 to 12
months. It is important to capitalize on
“critical or sensitive” periods of devel-
opment—more difficult to advance to
chewable foods if these developmental
milestones are not made in timely ways
(Illingworth Lister, 1964).
n Cup drinking before 12 months of
age (although that does not mean that
children have to be totally weaned
from breast- or bottle-feeding). Some
children may continue to breastfeed
directly or take bottles. The American
Academy of Pediatrics (2018) recom-
mends eliminating nighttime eating
and drinking by 12 months of age and
weaning from bottle-feeding before

18 months of age (https://www.aap.org/
en-us/about-the-aap/aap-press-room/
aap-press-room-media-center/Pages/
Weaning-from-the-Bottle.aspx).
Efficiencies With Textures
and Amounts/Volumes
of Food and Liquid
Differences in efficiency with varied tex-
tures and different amounts per bite or sip
should be noted. Initial presentations of
food and liquid should be in small amounts,
usually about 1/3 teaspoon or 1 to 2 cc, likely
via spoon. For children who are reported
to resist spoon-feeding, a dry spoon may
be the best way to facilitate acceptance in
nonstressful ways before presenting a spoon
with food. Acceptance is the foundation for
all oral feeding. Acceptance sets the stage for
“testing” options with every change made in
small steps in one dimension at a time. The
clinical evaluation incorporates therapeutic
trials that are described in more detail in
Chapter 9.
Many children with disabilities seen
for clinical feeding evaluations are eating a
pureed diet because they have been previ-
ously diagnosed with oropharyngeal dys-
phagia. History may include documented
aspiration or suspected aspiration. They
may have had unexplained difficulty tran-
sitioning to expand textures. Clinicians
always have to remember that it is not
possible to delineate pharyngeal swallow-
ing during a bedside or clinic assessment.
Inferences can be made about pharyngeal
functioning that may aid in determining the
need for an instrumental swallow evalua-
tion following the clinical session.
The first food offered during a clinic
assessment is usually the texture reported to
be the easiest for the child. For children who
have not been introduced to liquids, gradual
changes can be made from pureed foods to
liquids. As a first step, liquids may be pre-
sented on a spoon, as the feeder can control
the amount more precisely than with a cup.
A thin puree or thickened liquid may be
used when the child shows slow initiation
of tongue action and a delayed swallow, or
if there are questions about aspiration of liq-
uids. For children who take liquids, liquid
intake should be observed with the familiar
feeder presenting liquid as the child takes
at home and in other settings, e.g., child
care center. Depending on the child, com-
parisons may be made between patterns the
child uses to swallow thin and thick liquids
and patterns used for single sips versus con-
secutive swallows from an open cup or cup
with a lid that does not have flow control.
After observing typical drinking patterns,
the clinician may make some therapeutic
changes that will aid in management rec-
ommendations (Chapter 9).
Observations of Munching
and/or Chewing
Caregivers should bring chewable food if a
child has some experience with finger foods
(e.g., easily dissolvable and/or soft solids)
or they think a child should be eating solid
foods, but the child has either not shown
readiness or has been refusing to take solids.
Some children have a history of gagging or
vomiting with solid food. A child may be
assessed with chewable food even if it is a
new experience. There is evidence that solid
foods need to be introduced at appropriate
times even with children who are develop-
mentally delayed. The longer the delay, the
more difficult it may become for these chil-
dren to accept texture changes (Illingworth
Lister, 1964). Food choices are made to
minimize the risk for choking. The National
Dysphagia Diet provides descriptions for
the size and shape of foods (Cichero et al.,

2017). These distinctions appear to be con-
tained within level 6 (soft and bite sized)
for transitional foods on the International
Dysphagia Diet Standardization Initiative
(IDDSI, http://www.swallowstudy.com/id
dsi-resources/). Strip rectangle shapes can
be placed on molar surfaces to facilitate ver-
tical jaw motions for munching that in turn
may facilitate chewing actions and poten-
tially decrease the risk of choking. These
shapes may include dry snack foods that
dissolve (e.g., puffs, some graham crackers,
dry cereal, or plain sugar cookies). Some of
these dry foods are often advertised by com-
mercial businesses as the initial finger foods
to offer older infants. These dry foods pro-
vide sensory feedback (e.g., “crunch” sounds
and tactile input) that may help advance the
chewing process. These foods may be easier
for a child to grasp, since they are not slip-
pery. Examples of foods that can typically be
formed into a bolus with limited munching
or chewing include, but are not limited to,
veggie strips, graham crackers, plain sugar
cookies, or semicooked carrots. Dime-sized
pieces of soft wet foods that mash easily (e.g.,
cooked carrots, potatoes, or avocados) may
also facilitate chewing, although caution is
urged with circular-shaped pieces, which do
place children at higher risk for gagging or
choking. In contrast to the dry foods, these
wet foods may be slippery and may not offer
the discrete sensory input when children
transfer pieces from the tongue to the teeth
or gums. Some children may use the tongue
to mash rather than chew these wet foods.
In general, as chewing is emerging, the risk
for choking or aspiration appears to be less
for foods that hold together compared to
foods that particulate or break into small
pieces (e.g., potato chips, pretzels, or dry
thin crackers) or those with skins or kernels
(e.g., hot dogs, corn, or peas). Caregivers
are urged to avoid small hard textures that
could be swallowed whole readily (e.g., pea-
nuts or solid hard candy) as they present a
high risk for choking. During assessment of
chewing, clinicians need to describe move-
ments of the lips, tongue, and jaw, as well
as how well foods are chewed and readied
for swallowing. Wilson and Green (2009)
conducted a kinematic study of mandibu-
lar movements with typically developing
children ages 9 to 30 months. Early chew-
ing involved poorly graded jaw movements,
and the efficiency of mastication improved
as the child learned to adapt mandibular
movements to the consistency of the vari-
ous boluses. A child may not be able to
contain the bolus with limited lip closure.
Lateral tongue movements may be observed
as the child moves the bolus from tongue to
molars or gums. During the evaluation, it
is recommended that the clinician prompt
the child to open his or her mouth after the
bolus is processed, if possible, to check for
food packed in the lateral or anterior sulci,
or for residue on oral structures.
Utensils and Effects
on Swallowing
Assessment of spoon-feeding helps the cli-
nician delineate tongue, jaw, and lip move-
ments. Familiar spoons or other utensils
should be used first, and then therapeutic
spoons can be introduced to note differ-
ences in function. These oral sensorimotor
motions may be exaggerated and thereby
lead to disruption of rhythm and organiza-
tion, both of which are critical to coordina-
tion of bolus transit and swallowing. Each
task should yield information to facilitate
identification of the structures involved
most prominently in the oral sequences
and how any incoordination differs from
the expected typical movement. Three
examples of exaggerated movements that
may interfere with oral feeding are tonic

bite, tongue thrust and retraction, and lip
retraction. Although they are described as
individual actions in a child, these nonfunc-
tional movements are interrelated, which
makes it more challenging for clinicians
and parents to make management decisions.
Tonic Bite
A child with a tonic bite clamps down on a
spoon intermittently as it enters the mouth.
Once a tonic bite has been initiated, the child
may not be able to release the bite sponta-
neously. The spoon should not be tugged
in attempt to remove it from the mouth,
since that action by a feeder could hurt the
child’s teeth and, in fact, the bite reflex is
more likely to become more entrenched.
This action disrupts the rhythm and leads
to overall disorganization. Likewise, the
child with a tonic bite may clamp down on
the fingers while playing apart from eating
and drinking. As the child has more nega-
tive experiences, he or she is likely to do less
oral stimulation. In turn, avoidance of oral
stimulation may increase. The combina-
tion of lack of pleasurable oral stimulation
and reduced stimulation may increase the
possibility of oral defensiveness. When a
spoon or fingers cannot be pulled out of the
mouth easily, the child may be more likely
to increase biting. Two potential strategies
that may “break” a tonic bite are pushing
up against the mandible or gently messaging
the temporomandibular joint (Chapter 9).
Tongue Thrust and Retraction
Tongue thrust and tongue retraction both
interfere with efficient handling of food.
Both tongue patterns indicate that the child
is not using the tongue to organize food
to form a bolus and propel it posteriorly to
initiate a timely swallow. A tongue thrust
interferes with placement of the food in the
mouth, often resulting in spillage of liquid
or food out of the mouth before lip closure.
It can also interfere with the child’s ability
to suck, chew, and swallow. Tongue retrac-
tion is most noticeable “at rest” because the
tongue is held posterior in the oral cavity.
This retraction may be mistaken for tongue
thrust because in both instances food gets
pushed out of the oral cavity. The difference
is that with tongue retraction, food can be
placed into the front of the mouth easily, but
not necessarily on the tongue, rather in the
anterior sulcus or under the tongue. Once
the mouth closes, the tongue has to move
forward from its posterior position to scoop
the food, which in turn is often pushed out
of the mouth.
Lip Retraction
Lip retraction may be associated with sen-
sory defensiveness or increased tone and
usually results in excessive tension in the
lips. A child with lip retraction has diffi-
culty removing food from a spoon, drinking
from a cup, and sucking via nipple or straw.
Sensory defensiveness may be exhibited in
response to touch around or in the mouth.
Light touch, which is ticklish, often results
in strong pulling away from the stimulus.
Sensory defensiveness and increased tone
may result in abnormal movements or pos-
turing throughout the entire body.
An important goal during the assess-
ment is to determine whether the child
demonstrates a feeding disorder with or
without a primary swallowing deficit (dys-
phagia). Signs of bolus formation and oral
transit can usually be determined during a
clinical observation. However, pharyngeal
swallow function can only be inferred by
clinical observation of feeding. Instrumental

swallow examinations are needed to delin-
eate pharyngeal and upper esophageal swal-
lowing function. Predictions of swallow
deficits can help in planning an instrumen-
tal evaluation. It is important to determine
which evaluation (VFSS or FEES) has the
potential to answer the pertinent questions
(Table 7–10 and Chapter 8). For example,
the clinician can look into the oral cavity
and see material in the anterior or lateral
Table 7–10. Signs and Symptoms of Swallowing Deficits in Bolus Formation, Oral and
Pharyngeal Swallow Function With Possible Treatment Options
Swallow Function Sign or Symptom Possible Treatment Optionsa
Oral preparatory or
bolus formation
Food falls out of mouth Posture and seating
Pooling in anterior sulci Sensory aspects of food
Lack of tongue action to form
bolus
Lip closure
Rotary tongue and jaw action
Lack of chewing Sensory aspects of food
Oral transit Pooling in lateral sulci Lip closure and buccal tension
Food pushed out of mouth Tongue exercises to reduce
thrusting
Slow bolus formation Tongue manipulation
Piecemeal deglutition Lateral tongue action and rotary
jaw action
Delayed swallow Initiate tongue action quickly
Pharyngeal Pooling in valleculae and
pyriform sinuses
Posture and seating; improve
timing of swallow production
Residue in pharyngeal recesses
after swallow
Multiple swallows per bite;
alternate textures
Gurgly voice quality Improve vocal fold closure with
voice therapy
Aspiration on liquids, safe for
thicker textures
Change utensils (e.g., after nipple
flow rate, try spoon for discrete
boluses) or thicken liquids.
Aspiration on paste, safe with
liquids
Make food thinner texture
Choking on mixed textures in
the same bite
Make each bite of a consistent
texture; alternate per bite
Swallow delayed a few seconds
for best texture
Nonoral feedings
Aspiration for all textures
(frequent)
Nonoral feeding; oral stimulation
without food except for tastes
a
These treatment options are explained in detail in Chapter 9.

sulci before, during, or after a swallow. Par-
tial swallow of a bolus may result in residual
in the oral cavity. Tongue movements and
jaw action can be viewed directly at times,
but as soon as the lips are brought together,
the clinician can only make inferences. Lip
closure, or lack of closure, and liquid or food
loss out of the mouth can be seen. Bolus
formation time and oral skills, particularly
for chewing, can be noted (Gisel, 1991).
Children with CP typically show delays
in expanding textures. Chewing function
can be a target for observation and use of
an instrument developed by Serel Arslan,
Demir, Barak-Dolgun, and Karaduman
(2016). These children often take longer to
chew hard, solid food than typical children
(e.g., Gisel, Alphonce, Ramsay, 2000). As
reviewed by Faulks and colleagues (Faulks,
Collado, Mazille, Veyrune, Hennequuin,
2008; Faulks, Mazille, Collado, Veyrune,
Hennequin, 2008), children with Down
syndrome present with chewing prob-
lems because of an interplay between their
genetic predisposition and the interface
between muscle tone and skeletal develop-
ment. Interested readers are referred to the
articles by Faulks and colleagues (Faulks,
Collado, et al., 2008; Faulks, Mazille, et al.,
2008). The number of swallows per bolus
may be estimated, but not measured, when
the clinician can see, feel, or hear an audible
swallow at times. One may estimate the time
before seeing laryngeal elevation/excursion
that may provide a clue about swallow ini-
tiation, but it is not possible to be sure that
a pharyngeal swallow has occurred even
with obvious laryngeal excursion. Head and
neck position changes and facial grimacing
are visible but not definitive to delineate
swallow function.
Pharyngeal problems can be inferred
by noting a significant delay in initiation of
a swallow, gurgly voice quality, cough, and
increased respiratory effort or respiratory
distress. The longer the delay in production
of a swallow, the more consistent a gurgly
voice quality, and the more swallows needed
per bolus with any respiratory distress, the
greater the probability of pharyngeal swal-
low problems. Such problems should be
delineated objectively with an instrumen-
tal swallow evaluation, most commonly a
videofluoroscopic swallow study (VFSS) or
fiberoptic endoscopic examination of swal-
lowing (FEES) (Chapter 8).
Feeding specialists need to differentiate
immature but essentially normal patterns,
from abnormal patterns. Recommenda-
tions for intervention as well as prognosis
for improvement differ between these two
findings. Children with immature oral
skills are easier to manage than those who
have abnormal patterns. However, many
children have a combination of immature
and abnormal patterns that result in more
challenging decision processes. Once feed-
ing observations are completed, clinicians
make decisions according to the multiple
factors learned from history and observed
in the session. These delayed and/or devi-
ant patterns may be distinguishable in suck–
swallow sequencing, jaw control or stability,
tongue mobility, lip closure, dissociation of
tongue and jaw, and cheek movements dur-
ing drinking and chewing. Clinical feeding
assessment options can be found in the lit-
erature with a proliferation noted in recent
years. Thus, these authors opted not to
describe specific tools. Clinicians need to
evaluate for appropriateness, reliability, and
validity regarding any “standardized” tool.
Aversive responses may be noted in a
variety of ways to include, but not limited
to, food refusal with head turning away
from spoon or cup, hyperextension of the
head and neck, spitting food out of the
mouth, or clamping the lips tightly shut to
prevent food from getting into the mouth.
Unfortunately, caregivers and professionals

commonly see these activities as a behav-
ioral habit or sensory defensiveness. In
some instances, behavioral factors may be
primary, but one always must consider the
possibility of underling physiologic reasons
(e.g., esophagitis and constipation, to name
a few). The history and physical findings
frequently determine an underlying promi-
nent physiologic basis that may have been
prominent in the past and is carrying over to
the present. Because of the complex factors
involved with food refusal in most children,
team evaluations are particularly valuable.
Completion of the Clinical
Assessment and Follow-Up
Once the clinical assessment has been
completed, additional information may be
needed. Referrals may be made for various
tests (e.g., radiologic, metabolic, respiratory,
and other diagnostic studies). All profes-
sionals involved with any child and family,
whether the clinical evaluation was carried
out by a single professional or a team of pro-
fessionals, should compile findings from the
assessment(s) for optimal coordinated rec-
ommendations. Physician input is of utmost
importance in developing the management
plan for children with defined medical and
health risks.
Management options are discussed in
detail in Chapter 9. The options vary ac-
cording to the history, etiologies, and obser-
vations made during assessment. Some of
the first management modifications may
involve changes in seating and positioning.
Seating specialists play a critical role in the
selection and modification of appropriate
equipment to support the body and facili-
tate oral feeding.
Texturevariationsmaybemade.Amount
per bolus and timing of bolus presentations
can be altered. Utensils may be changed.
Oral sensorimotor practice may be carried
out with or without food. Children may
receive full oral feedings, a combination of
tube and oral feedings, or nutritional needs
may be met entirely by tube feeding. When
a child is fed by tube, an oral stimulation
program may be done with no food or with
very small amounts of food or liquid for
pleasurable practice and to facilitate pur-
poseful swallowing, but not with a focus
on feeding. Case studies that include the
integration of assessment and management
concerns are found in Chapter 9.
Assessment of Child With
a Tracheostomy Tube
Thorough assessment of oral feeding for
a child with a tracheostomy tube may be
complicated depending on the status of the
upper airway, pulmonary status, gastroin-
testinal tract, tracheostomy tube size, and
whether a child can tolerate a speaking valve.
The presence of a speaking valve (Passy-
Muir speaking valve) in young children was
found to improve residue in the pyriform
sinuses but did not decrease aspiration or
laryngeal penetration (Ongkasuwan et al.,
2014). The degree and type of difficulty
with oral feeding relate most closely to the
underlying reasons for the placement of the
tracheostomy tube, delayed introduction of
oral feeding because of prolonged intuba-
tion, and the degree of respiratory support
required by the infant (Joseph, Evitts, Bay-
ley, Tulenko, 2017). A tracheostomy tube
may restrict laryngeal elevation and thus
interfere with swallowing. Long-term tra-
cheostomy may affect swallowing in young
children (Abraham Wolf, 2000). Young
infants may have better tolerance than older
children because the infant larynx is high
in the neck, which makes the presence of a
tracheostomy tube less likely to have a neg-

ative impact on this aspect of swallowing.
Airway protection status must be verified
before a child is given any liquid or food.
Colored food or liquid is not sufficiently
sensitive to screen for aspiration. Accurate
diagnosis requires instrumental testing
via endoscopy or fluoroscopy. Endoscopy
is the procedure of choice when concerns
are raised for the safety of swallowing oral
secretions and mucous. When oral secre-
tions are suctioned from the tracheostomy
tube, it is clear that aspiration has occurred.
Any liquid or food taken orally that is suc-
tioned from the trachea or visualized at the
stoma should prompt feeders to pursue fur-
ther investigation. Oral feeding may need to
be discontinued for a short term until fur-
ther investigation is carried out (Chapter 4).
Clinical feeding observation and follow-up
instrumental examinations provide the
most comprehensive information as a basis
for formulation of a treatment plan.
Assessment With Children
Exhibiting Primarily Behavior
or Sensory-Related Problems
See Chapter 13 for assessment and manage-
ment. Readers are reminded that the physi-
cal and physiologic underpinnings to food
refusal are real and need to be delineated
before behavior and/or sensory focused
interventions can be carried out.
Conclusion
The clinical evaluation of infants and chil-
is an ongoing process. The initial evalua-
tion lays the foundation for establishing
functional baselines of feeding/swallowing
function. Decisions can then be appropri-
ate and timely. If additional information is
needed, further testing is carried out. From
the initial evaluation, goals are developed
in collaboration with the family and child if
he or she is able to contribute. Goals need to
be reflective of the child and family’s values
and priorities, as well as health and devel-
opmental needs. Management plans can
then be formulated. Management options
are discussed in Chapter 9. Once manage-
ment decisions are made and intervention
processes are put in place, evaluation con-
tinues as an integral part for clinicians and
caregivers. As treatment continues, goals
are revised with the family, based on the
child’s progress, changes in health status, or
transition to a new environment (daycare,
school, etc.).
Monitoring health status over time is an
integral part of any and all types of interven-
tion. The child’s nutrition and growth are
priorities and must never be jeopardized as
feeding specialists, parents, and children all
work together to improve oral feeding skills
to whatever degree is possible. The Interna-
tional Classification of Functioning (WHO,
2001) and the Children and Youth Version
(WHO, ICF-CY, 2007) provide valuable
guidelines for clinicians to focus on func-
tioning and disability of all children as they
participate in daily mealtime experiences.
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Appendix 7–A
Example of Documentation for Feeding/Swallowing
Concerns, History, Physical Examination, and
Observations During Feeding/Swallowing Assessment
DEMOGRAPHIC INFORMATION
Name: Date of Birth:
Medical Record Number: Date of Evaluation:
Referring Source: Chronological Age:
Clinician: Corrected Age (when appropriate):
Reason for referral and caregiver’s concerns:

Diagnoses:
Put a (3) to indicate presence of any factor; describe briefly. NA = not applicable
MEDICAL HISTORY
History is notable for:
Birth History:
Full term/Preterm: Gestational age: ______________ Birth weight: ______________
Apgar scores: 1 minute _____ 5 minutes _____ 10 minutes _____
Problems with the pregnancy or delivery:
Maternal infection:
Gestational diabetes:
Polyhydramnios:
Substance abuse:
Other complications:
Intubation: ______________________________________
NICU admission: ______________________________________
Respiratory problems: ______________________________________
Supplemental ventilation: CPAP _________ HFNC ____ Liters/min
Supplemental O2: ________ Liters/min
Room air: Age when weaned _______________
Newborn hearing screen reported: passed failed
Other pertinent problems: _____________________________________

Surgical History: ________________________________________
Planned surgeries: ___________________________
Pulmonary History/Current Concerns:
Currently followed by Dr. *** for:
Past history:
Active concerns:
Pulmonary symptoms are/are not controlled
History of asthma
Medications for wheezing or asthma

Emergency room visits or hospitalizations over the past *** months due to
pulmonary concerns
History of recurrent pulmonary infections
Recurrent respiratory distress or cyanosis
History of BRUE (brief resolved unexplained event). How many _____ When: _________
Supplemental oxygen or ventilation requirements: __________________________
Pertinent evaluations: __________________________
Gastrointestinal History/Current Concerns:
Past history:
Active concerns:
GI symptoms are/are not controlled
History of G-tube with/without a fundoplication
History of growth problems: __________________
History of reflux. Symptoms of reflux include: __________________________
Gagging or emesis: During feed After feed (at least 30 minutes)
History of constipation
History of diarrhea
Pertinent evaluations:
Otolaryngology History/Current Concerns:
Past history:
Active concerns:
Obstructive breathing problems:
Snoring:
Mouth breathing

Vocal fold status: ________________________________________
Tracheostomy Type: ______ Size: ______ Cuff: Inflated Deflated
Speaking valve: ______________________
Phonation: __________________________
Cardiology History/Current Concerns:
Past history:
Active concerns:
Surgical history:
Neurology History/Current Concerns:
Active concerns:
Genetics History/Current Concerns:
Active concerns:
Allergies:
Current therapies:
Family history:
Syndromes
Consanguinity
Other: _________________________
Social history/exposures:
Lives with: Parents Grandparents Foster parent Other persons in the home
Stays at home Day care Attends school
Smokers at home: Inside Outside
Pets at home: Describe _________________________
Developmental/educational history:
Therapeutic intervention and foci of therapies
Early intervention: _______________________
Occupational therapist: _______________________
Physical therapist: _______________________

Special education: _______________________
Speech-language pathologist: _______________________
Psychologist/applied behavioral analysis (ABA) services: ____________________
Individualized Education Program (IEP): ________________________
Therapies focused on feeding/swallowing: ________________________
When: _______________________
Where: _______________________
Child’s response: _______________________
Caregiver’s response: _______________________
Miscellaneous comments: ______________________
Approximate developmental skill level: _______________________________________
When Gross motor Fine motor Cognitive Speech/language
Date Receptive:
Expressive:
FEEDING/SWALLOWING HISTORY
Previous swallowing related or instrumental evaluations:
Upper GI: Date: ______ Findings:
Videofluoroscopic swallow study: Date: ______ Findings:
Fiberoptic endoscopic evaluation of swallowing: Date: ______ Findings:
INFANTS
Feeding/swallowing problems: __________________________
Diet (per 24-hour day):
Quantity of liquid (oz/cc)
Dietary supplements: ___________________________
Feeding: Oral:
Breast Bottle (_____ nipple, formula _____ expressed breast milk)
Tube: Type: __________ Schedule: _________________
Textures in oral diet
Liquid ( thin: __________ thick: _____________________________)
Utensils:
Bottle and nipple:
Pacifier use:

Feeding schedule/routines:
Duration: 20 minutes 30–40 minutes 45 minutes
Intervals: 2 hours 3 hours 4 hours Other ________________
Location: Cradled in arms Crib or isolette Infant seat
Position: Semi-upright Prone Side-lying ( Elevated) Upright Swaddled
Feeders:
Primary feeder:
Multiple feeders:
Feeders outside home:
Child’s response to feeders is variable:
OLDER BABIES AND CHILDREN
Feeding/swallowing problems: __________________________
Diet (per 24-hour day):
Quantity of liquid (oz/cc)
Dietary supplements: ___________________________
Quantity of food
Feeding: Oral:
Tube: Type __________ Schedule: _________________
Textures in oral diet
Liquid ( thin: __________ thick: _____________________________)
Pureed foods (smooth):
Ground textures:
Lumpy or chunky textures:
Solid (regular table food):
Utensils:
Bottle and nipple Cup ( Lid Spout Open) Straw
Spoon Fingers Fork
Pacifier use:
Response to brushing of gums/teeth:
Feeding schedule/routines:
Duration: 20 minutes 30–40 minutes 45 minutes
Intervals: 2 hours 3 hours 4 hours Other ________________
Meals:
Snacks:
No schedule, child grazes:
Awakens during the night feed:

Feeders:
Primary feeder:
Multiple feeders:
Feeders outside home:
Child’s response to feeders is variable:
Mealtime environment, preferences, and behaviors or adaptations to encourage feeding:
Location of meals:
At home:
School:
Walking around or no specific location:
Other:
Typical seating for meals/snacks:
Held in caregiver’s lap Infant seat or stroller High chair/regular chair
Adapted chair Custom stroller Wheelchair Other ______________
Comments: Seating meets child’s needs Needs adaptations New chair is needed
Comments:
Preferences:
Liquid temperature preferences: Warm Cold Room temperature
Appetite: Good Inconsistent Poor
Preferred liquids:
Liquid temperature preferences: Warm Cold Room temperature
Preferred foods:
Food temperature preferences: Warm Cold Room temperature
Behaviors or adaptations to encourage feeding:
Distractions: Television/videos Music Toys Other: _____________
Nighttime feedings
DEGREE OF INDEPENDENT FEEDING (AS DEVELOPMENTALLY APPROPRIATE)
Bottle Cup/Straw Spoon/Pouch Finger Food Fork
Type:
Nipple:
Type:
Dependent
Assisted
Supervised
Independent

Describe child’s level of autonomy:
Caregiver provides choices
Child indicates choices
Child influences pace of meal
BASELINE OBSERVATIONS
Level of arousal and alertness:
Sustained for at least 10 minutes
Intermittent and fluctuating
Falls asleep within 4–5 minutes
Lethargic
State:
Usually calm, quiet
Infrequent irritability and calms easily
Frequent irritability and calms with holding
Frequent irritability and difficult to calm
Position at rest:
Prone Supine Side-lying ( Elevated)
Sitting: Independent Supported
Posture and symmetry:
Head/neck posture Flexion Hyperextension Asymmetry
Trunk asymmetry Limb asymmetry
Variable: _____________________
Tone normal Hypertonicity Hypotonicity Variable
Proximal stability: Adequate Deficient (Location: trunk pelvis shoulder)
Distal mobility: Adequate Deficient (Location: arms/hands legs)
Airway status:
No problem
Airway noises: Stridor Stertor
Dusky spells: with feeding apart from feeding
Mouth breathing
Supplemental oxygen dependent ( nasal cannula)

Tracheostomy: History/Current Concerns

Drooling (saliva spillage):
Developmentally appropriate or teething
Frequency: Seldom Variable Frequent Constant
Amount: Minimal Moderate Severe Profuse
Extent: To lip To chin To clothes To table or other surfaces
Impact: Bib or clothing changes each day: How many
Awareness: High Occasional Never
Communication:
Preverbal Nonverbal Communicates with Signs Communication System
Verbal: Intelligible Babbles Vowel vocalizations only
Vocal quality: normal Abnormal Aphonic
Breathy Hoarse Shrill Hypernasal
Gurgly Weak Hyponasal
Pitch: normal High Low
Loudness: Volume: normal Weak or soft Too loud
PHYSICAL EXAMINATION OF STRUCTURES AND ORAL SENSORIMOTOR
FUNCTION: CHECK (3) ALL ADDITIONAL OBSERVATIONS THAT APPLY
Face and mouth:
Facial Symmetry Asymmetry
Mandible: Normal Micrognathia Retrognathia
Cheek tone: Normal Reduced
Lips at rest: Normal Closed Lips apart Lip tie
Lip movement: Normal Retraction Pursing
Ankyloglossia: Anterior Posterior
Tongue:
Configuration:
Normal Soft (hypotonic) Contracted (hypertonic) Atrophic borders
Movements:
Normal Symmetrical Protrusion (midline or protrudes to one side)
Elevation Lateralization Rapid movements or fasciculations
Hard palate: Normal High arch Narrow Cleft
Soft palate: Normal Cleft Bifid
Elevation/retraction with phonation
Velopharyngeal approximation: ______
Tonsils: Size ___________________
Jaw: Normal stability Instability Trismus

Reflexes and nonnutritive sucking:
Response to stroking around mouth: Eager Inconsistent
Rooting when stroked near corners of mouth: Always Inconsistent Never
nonnutritive suck/swallow: Coordinated Incoordinated Not elicited
Sucking on little finger: Rhythmic Dysrhythmic
Gagging: Present Diminished Prolonged Absent
Protective or defensive responses—upon command or with touch (no food or liquid):
Swallow
Cough
Gag
EXAMINATION OF STRUCTURES AND ORAL SENSORIMOTOR
FUNCTION DURING FEEDING/SWALLOWING ASSESSMENT
Interaction with primary feeder with infant/child. Describe:
Communication/interaction
Positioning
Utensils
Amount per meal
Length of meal (minutes)
Avoidance/refusal
CLINIC/BEDSIDE FEEDING/SWALLOWING ASSESSMENT
Liquids
Type/utensil
Consumed
Observations
Purees
Type/utensil
Consumed
Observations
Solids
Type/utensil
Consumed
Observations

CHECK (3) ALL ADDITIONAL OBSERVATIONS DURING FEEDING THAT APPLY
Breastfeeding: Latch readily Poor latch
Bottle-feeding: Latch readily Poor latch
Lip movements: Normal Open Retraction Pursing
Loss of liquid: Minimal Moderate Significant
Tongue movements: Normal Lateral Thrusting
Suck/swallow/respiratory sequence: Normal Incoordinated
Suckling bursts: Appropriate pauses No pause
Flow rate (bottle): Normal (bubbles with each one to two sucks in “regular” bottle)
Poor
Laryngeal elevation/excursion: During swallow Absent
Pocketing of food/liquid: Cheeks Front of mouth
Nasopharyngeal backflow: Liquid only Food
Changes in cardiopulmonary function
Noisy breathing
Chest retractions
Inconsistent rate of breathing or dysrhythmia
Nasal obstruction
Systemic changes: Bradycardia Apnea Cyanosis
Desaturation (usually measured by pulse oximetry)
Coughing Choking Gagging Spitting
Emesis (vomiting): During feeding Apart from feeding
Spoon-feeding: Sucks food off spoon Brings lips together around the spoon
Upper lip active Upper lip no movement
Chewing: Suck–swallow Munching Rotary jaw Lateral tongue Abnormal
Mandible: Vertical movement Rotary movement
Straw-drinking: Normal lip seal Inadequate seal ______ Loss of liquid ______
Discrete sips ≥1 liquid swallows ______
Posture/seating during bedside/clinical evaluation:
Associated movements
Arching back, neck, or head
Squirming or withdrawing
Falling asleep
Other
Alert for entire feed Lethargy noted

Time to finish feeding: 30 minutes 30 minutes Feeding not finished
Amount of intake
Modifications during assessment:
Type
Response
CLINIC ASSESSMENT
Feeding/swallowing disorder may include, be related to, or contribute to problems
with: (Check [3] all that apply)
Airway protection
Nutrition or growth compromise, or gastrointestinal tract
Oral sensorimotor skills
Behavioral responses to mealtimes
Environmental factors (e.g., stress or inconsistent expectations)
Strengths:
Challenges:
PLANS/RECOMMENDATIONS
Oral feeding, tastes, sensorimotor
Oral feeding without modifications or restrictions
Oral feeding with modifications
Oral tastes for pleasure: ______________________
Nonoral feeding with nonnutritive oral stimulation
Other evaluations
Pediatric specialty services: Type:
Instrumental swallowing evaluation: Type:
Other:
Follow-up and interventions (see Chapter 9)

331
Instrumental Evaluation
of Swallowing
Maureen A. Lefton-Greif, Joan C. Arvedson,
Robert Chun, and David C. Gregg
Summary
Evaluation of swallowing and feeding often
requires the use of additional studies that
focus on various functional and structural
aspects of swallowing that are not visible on
clinical examination. Multiple instrumen-
tal modalities have been developed to help
assess swallowing function in children that
may be carried out directly or indirectly.
The three most commonly used studies that
visualize the portions of the oropharyngeal
mechanism are the videofluoroscopic swal-
low study (VFSS), fiberoptic endoscopic
evaluation of swallowing (FEES), and ultra-
sound (US) imaging of swallowing.
Clinical evaluation is of paramount im-
portance and typically precedes an instru-
mental swallowing evaluation. Instrumental
evaluations are invaluable in answering spe-
cific questions as to the presence and extent
of swallowing dysfunction, safety for feed-
ing, and the relative contribution of various
structures or physiologic processes to swal-
lowing and feeding deficits, particularly
risks for aspiration. Therapeutic modifica-
tions are important components to be incor-
porated during the instrumental evaluation.
A description of each procedure is
followed by a discussion of its technical
performance. Special considerations in
the preparation for, or the interpretation
of, each study are described. The relative
advantages and disadvantages of each are
detailed. Finally, the pearls and pitfalls of
interpretation and clinical correlation are
discussed. Case studies highlight some of
these factors.
Introduction
Comprehensive evaluation is critical to the
individualized management for infants and
children with feeding/swallowing disorders
and frequently requires the use of special-
ized examinations that capture images of
the structures and functions of swallowing
physiology that are not visible on physical
examination. VFSS and FEES are the studies
most often used, with US used less routinely.
Each procedure has its proponents. All
procedures provide useful information for
the various clinical situations encountered
when infants and children have abnormal
(or suspected abnormal) swallowing and
feeding. Other instrumental procedures
used in the evaluation of pediatric patients
with dysphagia include upper gastroin-
testinal (UGI) study, scintiscan, 24-hour
8

dual-channel pH monitoring, multichan-
nel intraluminal impedance pH probe,
flexible fiberoptic nasopharyngolaryngos-
copy1
(FFNL), direct rigid laryngoscopy-
and bronchoscopy (DLB), esophagoscopy,
and esophagogastroduodenoscopy (EGD)
(Chapters 4 and 5). In addition, manometry
and electromyography (EMG) are useful in
some instances.
Clinical evaluation of oral sensorimotor
function and swallowing is aided immea-
surably by the additional objective informa-
tion provided in specific situations (Ameri-
can Speech-Language-Hearing Association,
2000; Arvedson Lefton-Greif, 2017; Lang-
more Logemann, 1991; Logemann, 1993).
Objective measures are particularly impor-
tant in evaluating the pharyngeal and upper
esophageal phases of swallowing in terms of
both structure and function not attainable
by clinical examination alone. An example
is the well-known fact that the risk for aspi-
ration is greatly increased in most children
with multiple disabilities, although many
have no observable clinical indications (e.g.,
coughing or choking), especially in chil-
dren with neurologic impairment (Arved-
son, Rogers, Buck, Smart, Msall, 1994).
Moreover, the risk of aspiration without
an observable response is extremely high
in children with dysphagia regardless of
the underlying condition (Arvedson et al.,
1994; Lefton-Greif, Carroll, Loughlin,
2006; Weir, McMahon, Taylor, Chang,
2011). Thus, the clinical evaluation is inad-
equate for many patients. Inaccurate or
incomplete information will render recom-
mended treatment strategies to be ineffec-
tive at best and potentially harmful at worst.
The instruments chosen for an evalu-
ation will depend on the anatomic areas
and functional processes that need to be
assessed, the questions that need to be
answered, and institutional availability, as
well as knowledge and experience of pro-
fessionals involved. Instrumental measure-
ment and scanning techniques are not used
in isolation but as part of a comprehensive
evaluation with a thorough clinical exami-
nation of feeding/swallowing. Several dif-
ferent procedures may be performed, each
of which may be useful for the evaluation of
oral, pharyngeal, laryngeal, upper esopha-
geal, and respiratory function related to
normal and abnormal swallowing. More of
these procedures have been developed ini-
tially for use with adults; however, data are
becoming increasingly available for their
use with children.
This chapter presents information related
to FEES, VFSS, and US. Each procedure’s
utility, indications for use, procedural con-
siderations, and, perhaps most importantly,
interpretation are discussed. It must be
stressed that each of these evaluations pro-
vides information that is complementary to
information from other evaluations and the
clinical evaluation. Hence, findings must be
considered as only one part of the global
evaluation. Importantly, each procedure
provides only a glimpse of the swallowing
mechanism and typically for a short period
of time (Figure 8–1).
Fiberoptic Endoscopic
Evaluation of Swallowing
Flexible nasopharyngoscopy uses a flex-
ible fiberoptic endoscope to view the upper
aerodigestive tract directly in infants and
children. The anatomic and physiologic
information gained for assessment of the
1
The terms flexible fiberoptic nasopharyngoscopy, flexible fiberoptic nasopharyngolaryngoscopy, and flexible
fiberoptic laryngoscopy are used interchangeably.

8. Instrumental Evaluation of Swallowing 333
hypopharynx and larynx is critical for
accurate diagnosis in many presentations
of pediatric dysphagia. Use of FEES was
first described by Langmore and colleagues
almost three decades ago (Langmore,
Schatz, Olsen, 1988, 1991).
The endoscopic method for evaluation
of swallowing provides information about
the events occurring immediately before
and immediately after the pharyngeal swal-
low. Hence, compared to videofluoroscopy
(discussed later), the FEES provides infor-
mation that is limited by a period of “white-
out” during the pharyngeal swallow.
Advances in digital video systems and
digital distal chip technology have revolu-
tionized the use of this tool as safe and highly
informative in both the diagnosis and treat-
ment of swallowing dysfunction in patients
of all ages, beginning with preterm infants
(e.g., Plaat, van der Laan, Wedman, Halmos,
Dikkers, 2014). In some instances, FEES
may be an adjunct to VFSS (Bastian, 1991).
Real-time simultaneous integration of FEES
with a VFSS from the same patient has been
facilitated by technology advances.
FEES Procedure
Optimally, FEES is best performed by a
team consisting of a pediatric otolaryn-
gologist and a speech-language pathologist
(SLP). The physician is skilled at passing
the flexible scope and has the comprehen-
sive knowledge base to assess the anatomic,
physiologic, and functional abnormali-
ties found in the nasal, pharyngeal, and
laryngeal regions. The SLP has specialized
knowledge and experience in swallowing
and communication and is able to focus
on the oral sensorimotor status of the child
and functional aspects of swallowing. This
interdisciplinary team approach capitalizes
on the expertise of professionals in these
two allied fields, thereby providing a more
Figure 8–1. Common factors that determine/modify the impact and management
of the feeding/swallowing dysfunction. (Source: Adapted from Lefton-Greif, M. A.,
McGrath-Morrow, S. A. [2007]. Deglutition and respiration: Development, coor-
dination, and practical implications. Seminars in Speech and Language, 28[3],
166–179.)

comprehensive evaluation of the child’s
swallowing ability (ASHA, 2016).
Children are placed in their typical
position for feeding. Infants may be held
by a caregiver in a typical feeding position.
They may be swaddled. The FFNL is passed
transnasally to view the pharynx and lar-
ynx. In general, a 2.4- to 3.2-mm nasopha-
ryngoscope will pass readily in most infants;
a 2.2-mm scope is commonly used in the
neonatal intensive care unit (NICU); a
3.4-mm nasopharyngoscope may be used in
older children. Fiberoptic scopes with distal
chip at the tip are 3.2 mm. Topical anesthe-
sia is used by some teams, taking great care
to limit the area anesthetized to the nasal
cavity so as not to interfere with swallow-
ing function. In some instances, the FFNL
is used without administration of topical
anesthesia to the nasal mucosa (Leder
Karas, 2000). When anesthesia is used, it is
given as a spray from an atomizer or on a
cotton pledget. A mixture of 2% tetracaine
and 1⁄2% neosynephrine or oxymetazo-
line (1:1 mixture) is delivered to the nose.
Tetracaine doses of 0.3 mg/kg are used for
children under the age of 4 years (Willging,
2000). After about 5 min, the FFNL can be
passed transnasally into the hypopharynx
while the child is held in the caregiver’s
lap or is sitting on a regular chair or in a
wheelchair (Figure 8–2). Alternatively, lido-
caine 2% jelly can be used to lubricate the
scope as well.
Figure 8–2. Child sitting and undergoing fiberoptic endoscopic
examination of swallowing (FEES). Otolaryngologist is passing
scope, and speech-language pathologist is presenting liquid.

In some children, swallowing is evalu-
ated first for handling of secretions. One or
two drops of food coloring are placed on the
tongue. This technique also is used for chil-
dren who seem unable to initiate a swallow
as inferred from visual observation. Of note,
increased production of secretions associ-
ated with crying may complicate interpre-
tation about what might happen when the
child is calm. Depending on the reasons for
the evaluation, if aspiration of oral secre-
tions is witnessed, the test may be termi-
nated. If the child can take food or liquid,
the SLP gives various food textures often
tinged with food coloring to the patient to
swallow. The entire examination is reviewed
online, and recorded digitally for documen-
tation and additional review in slow motion
and frame-by-frame.
The otolaryngologist positions the
scope to visualize the base of tongue, hypo-
pharynx, and larynx as the liquid or food is
swallowed (see Figure 8–2). One can visual-
ize the bolus passing over the tongue base to
the upper esophageal sphincter. Laryngeal
penetration can be observed as tinged (or
colored) liquid or food enters the laryngeal
vestibule and potentially to the level of the
vocal folds. Aspiration occurs when the
bolus goes below the true vocal folds. One
should observe whether the child responds
to the penetration or aspiration with a
cough or other attempted clearing action.
A unique feature of the FEES is that it dis-
plays the interface between respiration and
bolus passage. In addition, it provides views
that show the accumulation of secretions in
the pharynx and, hence, may provide useful
information about the sensorimotor swal-
low response in relation to secretions.
FEES with Sensory Testing
Sensory testing (ST) with a calibrated air
pulse to the posterior pharynx to assess
sensory thresholds was used in the past
(Aviv, Murry, Zschommler, Cohen,
Gartner, 2005; Hartnick, Miller, Hartley
Willging, 2000; Willging, Miller, Hogan,
Rudolph, 1996; Willging Thomp-
son, 2005). At the time of this publication,
instrumentation for FEES with ST is not
commercially available.
Although no longer available, FEES/ST
has provided important information about
sensory responses and thresholds in chil-
dren with dysphagia or at increased risk for
dysphagia. Elevated laryngopharyngeal sen-
sory thresholds were positively correlated
with a prior history of recurrent pneumo-
nia, neurologic disorders, and gastroesoph-
ageal reflux (Willging Thompson, 2005).
In addition, decreased laryngeal sensitivity
(i.e., elevated sensory thresholds) is shown
to result in poor clearance of secretions in
children with apnea. Decreased laryngeal
sensitivity may induce a prolonged glottis
closure event to prevent aspiration, which
may play a role in infant apnea (Thompson,
Rutter, Rudolph, Willging, Cotton, 2005).
Advantages and
Disadvantages of FEES
The advantages and disadvantages of FEES
are found in Table 8–1. The best results are
obtained when a child is basically coop-
erative. Examiner experience and patient
preparation are important factors in gain-
ing cooperation in children (Link, Willging,
Miller, Cotton, Rudolph, 2000; Willging,
2000). Children who are developmentally at
about 2 years and older can present chal-
lenges for cooperation, but it should be rare
that a study cannot be completed. Bedside
endoscopic swallow evaluations are per-
formed easily, and the system can be used
in the radiology suite. FEES is also useful for
children with limited or no oral intake when
there are often questions about handling of

secretions and concerns about the contribu-
tion of upper airway defects contributing to
the swallowing problems (see Chapter 4).
Interpretation:
Pearls and Pitfalls
Swallowing function parameters include
pharyngeal secretions, passive and active
movement of a bolus (or material) into the
pharynx, laryngeal penetration, aspiration,
pharyngeal residue, vocal fold mobility, and
gag response (Arvedson Lefton-Greif,
1998; Willging, 2000). FEES is more sensi-
tive than other tests in establishing persis-
tent residue and abnormal accumulation of
secretions in the pharynx. Particularly when
airway concerns are present, FEES is pref-
erable to VFSS to assess airway safety even
before introducing oral intake for a VFSS.
It bears repeating that examiner expe-
rience in a nonthreatening atmosphere is
important to achieve successful results. In
addition, patient and parental preparation
may be time consuming but well worth the
effort. Depending on the age of the child
and questions by caregivers, preparation
may require additional time to discuss the
procedure with child and family, and for
some children, time to play to minimize
any anxiety associated with the procedure.
Adequate topical anesthesia appears
helpful for some children to participate in
the procedure. Minimal to no pain or dis-
comfort enhances the child’s participation
with trust in the physician, which in turn
improves the quality and reliability/validity
of the findings. When anesthesia is given
carefully so that it does not anesthetize the
pharynx, it should not compromise either
the airway or the results. With FEES as well
Table 8–1. Advantages and Disadvantages of Fiberoptic Endoscopic Evaluation
of Swallowing (FEES)
Advantages Can perform at bedside
Position of patient is flexible and not critical to results
Observation of structure and function of hypopharynx and
larynx is possible
Can be used with infants who are breastfeeding
Investigates sensorimotor function of hypopharynx and larynx
No radiation exposure, can be repeated and study may take
as long as needed
Tests response to secretions
Disadvantages Incomplete examination of pharyngeal phase of swallow
because of “white-out”
Visualizes structures only immediately before or after a
pharyngeal swallow
Cannot assess oral or esophageal phases of swallow
Unable to evaluate coordination of pharyngeal motility with
tongue action, laryngeal elevation or excursion, and upper
esophageal function
Minimally invasive (potential nosebleeds)

as VFSS and all medical procedures, find-
ings when patients are stressed and unco-
operative must be interpreted with caution.
The most informative evaluations mimic
the usual swallowing pattern for the patient.
Nonetheless, noncooperative children and
of course infants can be studied with a mod-
icum of information to be gained.
FEES is performed at bedside, includ-
ing in the NICU or in a clinic, and does not
require that children be taken to a radiology
suite. In the NICU, FEES can be performed
with infants who are breastfeeding (Wil-
lette, Molinaro, Thompson, Schroeder,
2016) and as a team assessment (Reynolds,
Carroll, Sturdivant, 2016). FEES is par-
ticularly helpful for children who have diffi-
culties transferring to alternate seating sys-
tems, such as those with severe scoliosis or
kyphosis. These children cannot be exam-
ined easily in the radiology suite either.
Children with muscular dystrophy or other
neuromuscular conditions can be examined
in their typical feeding postures in whatever
seating systems are used at home and school
environments.
Videofluoroscopic
Swallow Study
Videofluoroscopy is the primary imaging
technique for detailed dynamic assessment
of oral, oropharyngeal, pharyngeal, and
upper esophageal phases of a swallow. VFSS
or modified barium swallow (MBS) study
are the two most common terms for swal-
lowing studies that use videofluoroscopic
imaging procedures. A comprehensive eval-
uation of esophageal and lower GI function
(i.e., an esophagram and upper gastrointes-
tinal [UGI] study) requires additional pro-
cedures by a radiologist. The esophagus is
only screened during the VFSS procedure.
VFSS is useful for diagnostic purposes and
to assist in management decisions. Dif-
ferences in handling a variety of textures,
assessment of facilitative and/or compen-
satory techniques, and reeducation proce-
dures are well known (Logemann, 1993;
Martin-Harris, Logemann, McMahon,
Schleicher, Sandidge, 2000).
VFSS is used as part of a comprehen-
sive diagnostic evaluation of infants, includ-
ing premature infants, and children with
suspected swallowing deficits. Although
somewhat of a misnomer, the VFSS is often
referred to as the “gold standard.” This is an
exaggeration because the findings reflect
only a brief window in time in a somewhat
artificial setting. Nonetheless, the informa-
tion obtained when the study is carried out
effectively with a cooperative patient is valu-
able as an important “piece of the puzzle.”
VFSS has been used widely since the
early 1980s (Logemann, 1983), and its use
has been increasing because of the increas-
ing number of children with swallowing
dysfunction (Arvedson, 2008; Lefton-Greif,
2008). The VFSS remains the most compre-
hensive examination for evaluation of pha-
ryngeal function in the swallowing process
and its interface with oral/oropharyngeal
transit and cervical esophageal function.
Cricopharyngeal opening, esophageal
motility, and transit time are screened
when a bolus is followed through the upper
esophageal sphincter (UES) and esophagus.
Radiologic information gained is listed in
Table 8–2 (Arvedson et al., 1994; Arvedson
Lefton-Greif, 1998; Lefton-Greif et al.,
2018; Nordin, Miles, Allen, 2017).
DespitetheincreasinguseofVFSS,ques-
tionshavebeenraisedaboutitsutilityrelative
to justification for the associated exposure to
ionizing radiation. Clinical utility has been
criticized because of the paucity of infor-
mation on standardization of procedures
(e.g., amount and order of presentations

with barium contrast) and outcomes from
the use of VFSS information in manage-
ment decisions. All fluoroscopic proce-
dures, including the VFSS, involve exposure
to ionizing radiation and its associated risks.
These concerns are addressed.
During a clinic or bedside feeding/
swallowing evaluation, clinicians are lim-
ited to observations of the signs of prob-
lems on which they base their inferences
about the interface between bolus flow and
aerodigestive tract function. Most children
are referred for VFSS because of known
or suspected swallowing dysfunction or
the presence of diagnostic conditions that
are associated with increased risk of swal-
lowing dysfunction (American College of
Radiology, 2017; see Table 1–1). Some chil-
dren present with signs that raise concerns
about the presence of dysphagia, although
no identifiable etiology is yet available to
explain the presence of the signs or reported
symptoms. Other children may present with
limited or no clinical evidence of dysphagia
but have risks based on previously estab-
lished diagnoses or past medical history, or
they may be exquisitely vulnerable to the
respiratory or nutritional consequences of
dysphagia (e.g., infants born preterm with
chronic lung disease associated with prema-
turity and suboptimal weight gain). Find-
ings in the clinical assessment may lead to a
medical order for a swallow study, but one
must not conclude that the mere presence
Table 8–2. Structural and Functional Information Gained From
Videofluoroscopic Swallow Study (VFSS)
Bolus formation and transfer in oral cavity
Velopharyngeal function, nasopharyngeal reflux, or pharyngonasal backflow
Hyoid bone anterior excursion and elevation
Laryngeal excursion and elevation
Laryngeal vestibule closure for airway protection
Coordination of laryngeal excursion/and movements in relation to pharyngeal
phase function
Pharyngeal motility
Presence of secretions and contrast material along tongue base, in the
valleculae, or in pyriform sinuses before initiation of pharyngeal swallows
Number of swallows necessary to clear material from oral cavity and
pharynx per bolus
Residue of secretions and contrast material along tongue base, in valleculae,
in pyriform sinuses, and along posterior pharyngeal wall after swallows
Presence and timing of aspiration in relation to swallows of varied textures
Response or lack of response to aspiration events
Temporal measures of bolus flow passage and physiologic attributes of
swallowing
• Oral transit time
• Pharyngeal transit time

of one or more of these signs and symp-
toms necessarily means that a VFSS should
be carried out. The information obtained
from the VFSS must result in diagnostic
clarity or impact current management rec-
ommendations. As an important reminder,
a VFSS cannot rule out “micro” aspiration
given that even people without dysphagia
are known to aspirate (Gleeson, Eggli,
Maxwell, 1997).
VFSS General Procedural
Guidelines
Findings from the clinical examination
(Chapter 7) are helpful in planning for
VFSS. The goal of VFSS is to obtain maxi-
mal pertinent information that defines oral,
pharyngeal, and upper esophageal swallow-
ing physiology in minimal time. Attention
needs to be paid to the child’s posture, posi-
tioning, and sensitivity to oral stimulation/
experiences (Arvedson Lefton-Greif,
1998; Hiorns Ryan, 2006; Zerilli, Stefans,
DiPietro, 1990). Other considerations
include the standardization and presenta-
tion of barium viscosities in relation to the
array of food textures in the child’s diet.
Current evidence suggests that barium
products do not simulate the characteristics
of liquid and food in the diets of infants and
children (Cichero, Nicholson Dodrill,
2011; Suzuki, Kondo, Sakmoto, Kimura
Matsumoto, 2016). Steps to ensure an opti-
mal examination include:
1. preparation of patients and caregivers
for VFSS;
2. attention to physical setup for VFSS
in pediatrics, including seating and
postural considerations;
3. use of standardized viscosities (diffi-
cult when liquid and food are brought
from home); and
4. coordination of procedures,
particularly with the radiologist and
radiology staff.
This chapter is not intended to be a pro-
cedural manual. It is intended to highlight
aspects of procedures that should provide
a basis for improved consistent approaches
from facility to facility locally, nationally,
and internationally, with a variety of pro-
fessionals. Readers interested in detailed
information can refer to Arvedson and
Lefton-Greif (1998).
Preparation of Patients and
Caregivers for VFSS
Caregivers should receive sufficient verbal
and written information prior to the VFSS
so they understand the reasons for the study,
the way in which it will be carried out, and
what information may be gained. Caregiv-
ers can then help to prepare the child for
optimal cooperation. Parents may be help-
ful in the radiology suite with the child; in
most instances, the presence of a familiar
feeder is critical for success of the study. Cli-
nicians need to work closely with the radiol-
ogy department in their facility to achieve
the best examinations possible.
Children should be awake, alert, and
hungry. Parents (possibly with nurses in
the case of inpatients) are encouraged to
withhold food and liquid for several hours
before the examination. Children who
are hungry and thirsty are more likely to
accept the barium-impregnated food and
liquid. However, children should not be so
hungry that fussiness results or metabolic
equilibrium is disrupted. The VFSS should
be scheduled as close as possible to a regu-
lar feeding time. Timely appointments and
a nonthreatening, playful atmosphere also
are helpful with young children. Pacifi-
ers, swaddling, or partial undressing may

soothe infants. Caregivers are encouraged
to bring familiar foods, containers, and
utensils. They also may bring favorite toys,
electronic devices (e.g., video games, iPad),
and other comfort items. Cooperative chil-
dren are essential for an interpretable study.
Fussy, crying children are at increased risk
for aspiration because of incoordination
of swallowing if they take food or liquid
while they are crying. Findings cannot be
interpreted reliably if a child’s responses
are not typical of behavior during routine
feeding times. Children who are lethargic
may also be at high risk for aspiration. How-
ever, children who are typically in a some-
what lethargic state while eating orally may
undergo VFSS so clinicians can assist with
optimizing management decisions.
Additional considerations are needed
when children are fed in part or totally by
nonoral or supplemental tube feedings.
Regardless of the type of feeding tube, VFSS
should be done only after the child demon-
strates some level of experience with food
or liquid orally, at the very least in the con-
text of oral sensorimotor/swallow practice.
Children who are nonoral feeders are usu-
ally referred for testing because questions
are raised as to the possibility of manage-
ment changes, such as the introduction to
oral feedings. In some instances, a FEES
examination may provide information for
these children to determine if oral senso-
rimotor practice can include tastes of food
or liquid that will help to prepare the child
for oral taste stimulation or a VFSS. Parents
are reminded that this sample of swallows
is strictly oral. A bolus of at least 1 to 2 cc,
reflecting the amount of saliva in a typi-
cal swallow, is usually adequate to screen
swallow function. An ability to consume
15 to 30 cc, more closely approximating an
actual feed, is preferred. Children who do
not accept even oral tastes are not likely to
complete a safe, valid, or reliable study.
A nasogastric (NG) tube may be
removed when a child has been taking oral
feeding perhaps 50% to 75% of total volumes
required each day and appears nearly ready
for total oral feeding. The effects of NG tubes
on swallowing physiology are not clearly
understood in infants and young children.
The presence of NG tubes may interfere with
nasal airflow in infants who are predomi-
nantly nasal breathers during bottle feeding,
and they may increase the probability of gas-
troesophageal reflux (GER). Studies about
the impact of orogastric (OG) and NG on
specific attributes of swallowing are equivo-
cal. Two studies showed no impact of feed-
ing tubes dwelling in the pharynx (either
OG on NG) on findings of aspiration during
VFSS or FEES (Leder Suiter, 2008; Leder,
Lazarus, Suiter, Acton, 2011); however,
another study showed slowing of swallow-
ing with the presence of NG tubes (Huggins,
Tuomi, Young, 1999). More information
is needed about the impact of feeding tubes
passed transnasally into the pharynx and
esophagus, particularly for infants and chil-
dren. There are additional considerations
for a nasoduodenal (ND) tube because it
needs to be reinserted under fluoroscopy.
Therefore, an ND tube is left in place for a
child who is clearly not ready to do major
oral feeding. Likewise, leaving an NG tube
in place may help with recommendations
for a child who is expected to continue with
long-term NG tube feedings following the
VFSS. In this case, the child is visualized in
the anticipated feeding situation.
Schedules may need to be adjusted for
medications that are given in relation to
mealtimes so they fit in the timing of the
VFSS (e.g., medications for seizure control
and gastroesophageal reflux). If medications
make a child lethargic, scheduling times
may need to be adjusted. Overall, caregiv-
ers should keep their children as close as
possible to regular routines.

Physical Setup for VFSS
Fluoroscopy is a radiologic technique that
permits real-time dynamic imaging of the
swallowing mechanism. Digital images are
viewed in real time during the examination
and recorded for slow motion and frame-
by-frame review after the examination.
These images become the permanent record
of the study. Basic equipment does not dif-
fer in relation to the age of the patient. The
equipment is low dose by design and con-
sists of (a) a standard, tiltable fluoroscopic
table, (b) an image intensifier tube that is
impacted by the x-ray beam and generates
the image, (c) a video monitor for real-time
viewing, and (d) a digital recorder con-
nected into the fluoroscopic equipment.
The digital recorder is typically con-
nected into the controls of the fluoroscopy
unit so when the radiologist activates the
fluoroscopic unit the images are recorded
simultaneously. The simultaneous activa-
tion of video and fluoroscopy unit allows for
accurate viewing with no gaps. Almost any
digital recorder can be used to record the
image from fluoroscopy. Every study should
include audio. If a microphone is not built
into the recording system, it should be read-
ily available and accessible for every study.
If a headset is needed for audio replay, it
should be stored with the microphone. The
simultaneous audio recording is essential
to record instructions, describe events by
the clinician, hear the patient’s responses
(particularly useful in relation to aspiration
timing and presence or absence of a cough),
and assess vocal quality (e.g., breathy, gur-
gly, or dysphonic).
In clinical research facilities, the basic
setup includes recording equipment with a
high-resolution frame-pause, slow-motion
forward and reverse, and single-frame
advance capability (Arvedson Lefton-
Greif, 1998; Lefton-Greif et al., 2018; Loge
mann, 1993). Accessory equipment includes
image counter to number each frame or
field of the study, character generator to
print patient identifying information on
the image, and a printer to produce a hard
copy of a select recorded frame. Digital
imaging provides clinicians with immedi-
ate accessibility to images for review and
interpretation (Goske et al., 2011). The
increased emphasis on efficacy of treatment
and outcomes behooves all clinicians to use
research thinking processes and problem-
solving skills for data collection.
Safety
Radiation safety must be a high prior-
ity (Jones, Kramer, Donner, 1985). Two
rules of reason can be applied to patients
undergoing radiation exposure for medi-
cal purposes (International Commission
on Radiological Protection, 2007; Tolbert,
1996). The first rule relates to medical
necessity, which considers the importance
of specific information required from an
imaging procedure. This rule supports the
earlier discussion about the critical need to
define the questions to be answered before
considering referral for VFSS. Consider-
ation must be given to the diagnostic util-
ity and accuracy of information from x-ray
studies relative to the yield from modalities
that do not involve exposure to ionizing
radiation (Isaiah Pereira, 2017).
The second rule relates to the principle
of keeping exposure levels “as low as rea-
sonably achievable (ALARA)” (Alzen
Benz-Bohm, 2011; Dorfman et al., 2011;
Strauss Kaste, 2006; Tolbert, 1996). In
all instances ALARA means limiting expo-
sures to that amount needed to achieve the
purpose of the procedure (Tolbert, 1996).
ALARA is especially important in children
because their tissues are particularly sensi-
tive to the effects of ionizing radiation and

their longer life spans may predispose them
to the development of cellular and tissue
damage effects (Alzen Benz-Bohm, 2011;
Dorfman et al., 2011; Furlow, 2011; Strauss
Kaste, 2006).
All procedures are carried out in ways
that minimize dose to the patient and others
in the fluoroscopy suite (Hayes et al., 2009;
Meisinger, Stahl, Andre, Kinney, Newton,
2016). Dose is the amount of radiant energy
that gets into the tissues of the body. The
risk of harm to the patient is dose depen-
dent. The entire population is exposed to
variable amounts of natural environmental
radiation. This exposure is from naturally
occurring sources such as radon gas and
radiation from the sun. Patient dose during
fluoroscopy can be minimized by keeping
the exposure time as short as possible (“flu-
oro-on” time) (Huda, 2009). In addition,
careful collimation of the beam to the area
of interest, and reduction in the number of
radiographs taken, if any, are all useful in
reducing radiation dose (Beck Gayler,
1990). Two other factors that determine
patient dose are the diameter of the image
intensifier used, as defined by the electronic
magnification mode, and the body region
being irradiated as well as the x-ray beam
projection (Huda, 2009).
The fluoroscopic image needs to encom-
pass the lips anteriorly, the palate superiorly,
the posterior pharyngeal wall posteriorly,
and the bifurcation of the airway and the
esophagus inferiorly. Collimation of the
beam to the area of interest can significantly
decrease exposure by limiting primary and
scatter radiation. The field should be coned
to keep the ocular lens out of the field and
ideally the thyroid gland. However, it is
impossible to exclude the thyroid gland
from the field of exposure with focus on the
pharynx and larynx. Radiologists are con-
stantly challenged by active moving children
and frequently must follow that moving
target with the imaging field. Magnifica-
tion should be kept to a minimum since it
exponentially increases radiation dose. Use
of the lowest level of magnification needed
for visualization of space between laryngeal
surface of the epiglottis and arytenoids is
advocated. The only practical way to mini-
mize the dose to this area is to limit the fluo-
roscopy time during each examination and
the number of examinations.
Every swallow study should be moni-
tored to conform to radiation safety stan-
dards and to minimize the duration of the
study, as well as the amount of surface area
exposed (Kim, Choi, Kim, 2013; Minhas
Frush, 2013). Consensus-driven updates
for practice and technical standards in
radiologic practices in the United States
are available from the American College of
Radiology (https://www.acr.org). Swallow
studies are limited to a dose of 125 (±0.64)
mrad/study, with a 10% reduction in that
level recommended for children less than 18
years of age (Kim et al., 2013). Appropriate
shielding should be used at all times (Huda,
2015; Leung, 2015; Sivit, 1990). Every radi-
ology department has a safety monitor who
is responsible for the safe use of radiation
and radioactive materials as well as regula-
tory compliance, and should provide infor-
mation on institutional guidelines. Careful
planning will assure that no unnecessary
time is spent with the child under fluoros-
copy. Regarding timing, Beck and Gayler
(1990) stated that initial diagnostic studies
should rarely exceed 2 min of “fluoro-on”
time. In our experience, most studies with
infants can be completed in 60 to 90 sec,
and in some cases the examination can be
performed in less time. Factors that con-
tribute to duration of exams include, but
are not limited to, age and cooperation of
the patient, the number of swallow presen-
tations, the experience of the examining
clinician and the operator of the fluoros-

copy equipment, and the complexity of the
information needed from the examination
(Arvedson Lefton-Greif, 2017; Weir et al.,
2007). Patient cooperation and caregiver
involvement can reduce the exposure time.
Even with the most difficult older patient,
clinicians are urged to keep total fluoros-
copy time to no more than 2 or 3 min with
rare exceptions. Therapeutic maneuvers
that include changes in head position or
evaluation of different bolus consistencies
and volumes take longer. Thus, therapeu-
tic maneuvers should be used sparingly
when needed for determination of utility
in making optimal recommendations for
management.
Adjustments in frame rate are used to
minimizeradiationexposure.Currently,con-
tinuous fluoroscopy, a fluoroscopic pulse rate
of 30 frames per second (fps) is considered
necessary/optimal for capturing swallow-
ing impairments (Arvedson Lefton-Greif,
1998; Cohen, 2009). Although lower frame
rates (12.5–25 fps) have been reported, data
suggest that these lower rates are inadequate
for detection of penetration and aspiration
events, which would likely be missed at less
than 30 fps (Cohen, 2008, 2009; Hender-
son, Miles, Holgate, Perryman, Allen,
2016; Weir et al., 2007). Research is needed
to determine the lowest frame rate needed
for obtaining reliable and valid findings,
which yield optimal clinical utility and the
best patient outcomes (Bonilha et al., 2013;
Nordin et al., 2017).
Seating and Positioning
Caregivers and clinicians work together to
achieve a typical feeding position for each
child. If the typical position is different
from an optimal position, the child should
be observed in both positions. In general,
the child’s posture should be one that attains
central alignment. No single definition of
optimal position can be stated because indi-
vidual exceptions may be needed. See Chap-
ters 7 and 9 for seating considerations. Com-
mercial seating and positioning options are
available for infants and children. Seating
possibilities for infants and children neces-
sarily are varied to meet the specific needs of
that child within limitations of the fluoros-
copy equipment that allow for transmission
of images to encompass oral, pharyngeal,
and upper esophageal phases of swallow-
ing (Figure 8–3). Radiopaque objects (e.g.,
metallic head supports, snaps, and zippers),
which are visible in the imaging field, must
be removed because they can obstruct views
of the structures of interest.
Given the space limitations between
the upright table and the fluoroscopy unit
in most pediatric radiology suites (typi-
cally 15–18 inches [38–46 cm]), children
are transferred into special seating arrange-
ments. Seating systems need to be posi-
tioned as close as possible to the imaging
equipment to maximize the quality of the
image and reduce the amount of image
magnification. Wheelchairs, even those for
small children, are usually too wide to fit
between the table and the fluoroscopic tube,
and they are too low to allow for viewing the
oral cavity and pharynx, and especially for
screening the esophagus. Portable fluoros-
copy units are used in some adult facilities
(e.g., long-term care facilities), but they may
or may not be appropriate for infants and
young children. C-arm units may be adapt-
able for older children who need to remain
in their own wheelchairs. Other adaptations
may be possible, depending on specification
of equipment in the radiology suite
Seating systems can be adapted in a vari-
ety of ways to meet the needs of most chil-
dren. Basic stability and central alignment
of the infant or child must be maintained
with whatever modifications are made.
A child with poor head and/or trunk control

may need additional stabilization to attain
neutral midline position. Rolled towels or
cushions can be placed alongside the head
or trunk, but not metallic materials, which
may obscure images of interest. The child
must tolerate any restrictions of movement
so that a calm state is maintained. Position-
ing changes may be needed during an eval-
uation when difficulties become apparent,
most likely when the child is moved from
the typical to the anticipated optimal posi-
tion. For example, a 6‑month‑old infant
Figure 8–3. A. A child seated between the upright fluoro-
scopic table and the image intensifier prior to videofluoro-
scopic swallow study (VFSS). B. A 9-week-old infant taking
thin liquid by bottle for VFSS in lateral view. Parent is present-
ing the bottle.
B
A

may take nipple feeds in a semireclined
position and moved into upright position
for spoon-feeding.
Optimal positions for eating may vary
considerably from one child to another.
Caregiver perceptions may also differ. Some
children may be supported more effectively
to maintain central alignment while semi-
reclined at an angle of approximately 35° to
45ο
than when in upright position. Infants
may be observed in a reclined position that
resembles that of nursing infants side-lying
or bottle-fed in a reclined position (New-
man, Cleveland, Blickman, Hillman,
Jaramillo, 1991). A side-lying position may
be necessary for a premature or very young
infant (Lau Smith, 2012) and for a child
with a unilateral vocal fold paralysis with
the nonparalyzed side down, or for a child
with severe muscle tone imbalance (Geyer
McGowan, 1995; Ward, 1984). This
position may not be helpful for bottle-fed
infants who are not feeding at home in side-
lying position. An infant with Pierre Robin
sequence may be able to bring the tongue
forward within the oral cavity for more effi-
cient sucking action and maintenance of
airway patency while in a modified prone
or semiupright side-lying position. It is best
to simulate the normal or desired feeding
position as closely as possible.
A caregiver typically needs to participate
in the feeding during VFSS procedures. In
rare instances, children resist separation to
such a degree that it may be necessary to
have the child seated on a caregiver’s lap.
Radiology department regulations may pro-
hibit that possibility. Decisions are made by
radiologists and the regulations of the facil-
ity. It is not possible for a caregiver to cradle
an infant in the arms because the caregiver
would block the pharyngeal view of the
infant. Creativity may be needed to make
the study successful with fearful children,
or those who have severe motor deficits. It
is also true that some children respond in
more cooperative ways when caregivers are
not in the radiology suite.
The success of the procedure depends
greatly on the most stable seating or posi-
tioningsystempossible(ArvedsonLefton-
Greif, 1998). Interpretation of the study will
have to be qualified if there are questions
about the adequacy of positioning. The
young child who walks into the radiology
suite, stands in place or sits happily, and
eagerly takes the food and liquid presented
is relatively rare.
Some children need additional prepara-
tion prior to this examination. A child with
a tracheostomy tube may need suctioning
before taking food and liquid. Suctioning
capabilities should be available through-
out the process. For a child using a speak-
ing valve, imaging most likely will pro-
vide the most complete information with
and without the valve. For a child who is
accustomed to oral stimulation before food
presentations, a brief practice period with
a caregiver or a clinician familiar to that
child may be helpful. Another child may
benefit from “activities” that diminish oral
defensiveness or tactile hypersensitivity. In
summary, adequate positioning and prepa-
ration are critical to the success of the VFSS.
Once the child is positioned appropriately
and appears ready to take food and liquid,
the procedure begins.
Carrying Out VFSS:
Guidelines and Techniques
Typically, VFSS is conducted by a team
that may vary in different medical settings
based on radiology department regulations
and availability of professionals. Clinical
practice should be based on the best data-
based evidence available. Clinicians need to
be aware of new evidence so they can adapt
their practices to obtain the most valid
and reliable data with the least exposure to
radiation for every infant and child. Medical

participation typically involves a radiolo-
gist, a radiology physician assistant (PA),
or another physician who is knowledgeable
regarding radiation safety and techniques,
an SLP (American College of Radiology,
2017; ASHA, 2016), and an x-ray technolo-
gist. The SLP must demonstrate appropriate
knowledge and skills to ensure that every
decision made will benefit every patient
who undergoes a VFSS (ASHA, 2016). In
some institutions, a team with a qualified
SLP and radiology technician may conduct
the study with consultation as needed from
the physician. With high-risk and medi-
cally fragile infants and children, immedi-
ate access to appropriate medical personnel
and equipment is essential. The radiologist
or PA operates the fluoroscopic unit, iden-
tifies structural abnormalities, and when
necessary, terminates a procedure that has
unacceptable risks to the patient’s health
and safety.
The basic technique for VFSS has been
described previously in detail (Arvedson
Lefton-Greif, 1998; Logemann, 1993). For
outpatient appointments, caregivers are
often asked to bring samples of food in the
child’s usual diet, and especially those food
textures for which they have the greatest
concern related to coughing and gagging,
increased time for feeding, or increased
need for suctioning. This process allows
for identification of foods and liquids that
can be ingested safely and where risks for
laryngeal penetration and aspiration may
become evident. Barium impregnated food
and liquid become the test materials, with
as close approximation as possible to foods
that normally appear in the diet. There is no
way that the use of barium products in food
and liquid can be considered the equivalent
of the usual food and liquids (Cichero et al.,
2011; Frazier et al., 2016; Strowd, Kyzima,
Pillsbury, Valley, Rubin, 2008). Although
every effort is made to assess the child in
the most normal or typical oral feeding
situation, there may be times when other
approaches are necessary, especially in
the presence of severe oral sensorimotor
dysfunction.
Barium sulfate products are used because
of their high molecular density that acts as a
positive contrast agent for radiographic pro-
cedures. Barium sulfate is effectively inert
and therefore not absorbed or metabolized
by the body. It is eliminated unchanged from
the body. Barium sulfate products should
not be used in patients with known or sus-
pected gastrointestinal tract perforation,
known or suspected colonic obstruction,
or hypersensitivity to barium sulfate for-
mulations. Caregivers should inform their
physician if the child is allergic to any drugs,
food, or if there has been any prior reaction
to barium sulfate products or dyes used for
radiology procedures. Commercial barium
sulfate materials should be latex-free and
acceptable for patients on specific diets (e.g.,
ketogenic). In our experience, barium com-
panies have been most helpful in identifying
ingredients for review of their acceptability
by physicians and dietitians.
Children who eat and drink varieties of
food and liquid usually get samples of liq-
uid, smooth and lumpy puree, and chew-
able food impregnated with barium sulfate.
Standardized barium products are commer-
cially available. Objectivity and standardiza-
tion of rheologic properties and viscosity are
needed to provide clear and consistent infor-
mation for comparisons between and within
patients and to create a common language for
the development of meaningful outcomes.
These factors have the potential for decreas-
ing lifetime radiation exposure by limiting
the need for multiple VFSS studies (Cichero,
Jackson, Halley, Murdoch, 2000; Cichero
et al., 2017; Lefton-Greif et al., 2018; Martin-
Harris, Humphries, Garand, 2017).
The common procedure is to start with
the thinnest liquid barium contrast first
with a controlled small volume, then to con-

secutive swallows by nipple, cup, or straw. It
is important that the findings not be com-
plicated by residue of thicker material in the
pharynx, which could be the case if a thicker
consistency is given first. The order of pre-
sentations may be altered to ensure some
degree of cooperation and in children who
are tactilely defensive, because they may
allow only a minimum number of bolus
presentations. Procedural adjustments are
made to meet the primary goal of obtain-
ing maximal information in minimal time.
The order of presentation of liquid and
food may change on the basis of history,
prior observations about how the child han-
dles various textures, and anticipated coop-
eration per caregiver recommendations.
Bolus size and timing of presentations can
also be varied because children’s abilities
may differ. Some children show improved
timing and coordination of oropharyngeal
swallowing with larger boluses when they
have only a brief pause between bolus pre-
sentations. Infants may be given liquid via
nipple of different viscosities if they dem-
onstrate laryngeal penetration or aspiration
before or during swallows when taking thin
liquid initially. When infants cannot pro-
duce rhythmic suck–swallow sequences suf-
ficient to extract enough liquid for observa-
tion of swallowing, approximately 1 to 2 ml
of liquid may be presented via syringe or
spoon. The choice of nipple is another con-
sideration that complicates interpretation
because of considerable variability in flow
rates of various nipples (McGrattan et al.,
2017; Pados, Park, Thoyre, Estrem Nix,
2015, 2016). Breastfeeding infants pres-
ent particular challenges because it is not
possible to duplicate the viscosity of breast
milk with barium contrast, even when using
a preprepared standard liquid or powder
mixed with breast milk which then must be
presented via bottle and nipple. Sufficient
intake of contrast is critical to obtaining
valid and reliable findings.
In older children, liquid may be pre-
sented first via spoon, and then, depending
on the questions and the skills of children,
larger quantities may be given via cup or
straw. Clinicians must remember that if
residue occurs with thicker material, visu-
alization of thin or very thin liquid boluses
may be compromised by the presence of
residue in the pharynx. Changing the order
of consistencies may make it difficult for
observers to determine the basis for any
aspiration events. Clinicians must be astute
observers and “online” decision-makers
with pertinent data regarding history and
current feeding status.
The SLP and radiologist make observa-
tions relating to timing of the swallow, coor-
dination in oral and pharyngeal phases of
the swallow, pharyngeal motility, presence
or absence of material in the pharyngeal
recesses before a swallow or residue in the
pharyngeal recesses after the swallow, and
esophageal transit time. Occurrence of aspi-
ration before, during, and/or after swallows
of varied textures is documented. It is also
important to document whether a child
responds to the aspiration with a cough or
some other observable action, attempts to
clear with a cough, or makes no response
(silent aspiration).
In some children, it is not uncommon
that aspiration may occur on the first one
or two swallows as they are getting orga-
nized. With additional swallows or dif-
ferent textures as they get “warmed up,”
improved timing and coordination with
less or no aspiration is seen. Other children
may show an increased frequency of aspira-
tion as the study progresses. Given that the
study samples only a few swallows when a
child has not eaten for at least a few hours,
it is not possible to evaluate the changes
that may occur over time or to simulate an
entire meal. Observations of feeding dur-
ing a clinic/bedside assessment should help
clinicians structure the tasks during the

VFSS. Clinicians must remember that the
clinical utility of the VFSS is dependent on
the relationship between swallow physiol-
ogy and bolus flow, and not just the pres-
ence or absence of penetration or aspiration
(Martin-Harris et al., 2000).
The lateral view gives the best view for
extrapolating useful temporal information
for most pediatric patients. It is also the
easiest and clearest view for detection of
aspiration. This lateral view permits obser-
vations of lips, tongue, palate, epiglottis,
laryngeal structures, and upper esopha-
gus (Figure 8–4). Pharyngeal motility can
be observed along with the opening of the
UES. Table 8–3 summarizes swallow func-
Figure 8–4. A. Lateral view of oral and pha-
ryngeal structures on presentation of bolus:
soft palate (P), vallecula (V), hyoid bone (H),
pyriform sinus (PS), esophagus (E), and tra-
chea (T). B. Lateral view focused on pha-
ryngeal swallow showing barium contrast
in valleculae (V) and pyriform sinuses (PS)
before swallow is initiated. Note: Fluoroscopic
imaging displays barium contrast in black more
commonly than in white. C. Lateral view show-
ing aspiration of liquid into the trachea (T) that
occurred after the swallow from residue in the
pyriform sinuses (PS) due to reduced pharyn-
geal motility. Note esophagus (E), hyoid bone
(H), and valleculae (V).
C
A B

tion and observations with potential radio-
logic abnormalities. Observations of bolus
preparation and containment include the
initiation, rhythmicity, and organization
of sucking in bottle-fed children (Lefton-
Greif et al., 2018). Barium contrast seen
in the valleculae or pyriform sinuses prior
to initiation of a pharyngeal swallow often
results from delayed initiation and, in some
instances, reduced tongue base retraction
and pharyngeal motility. Stasis and resi-
due may be texture related in some chil-
dren. Oral and pharyngeal transit times
can be measured. Nasopharyngeal reflux
Table 8–3. Swallow Function and Observations With Potential Radiologic Abnormalities
Swallow Function Observations Potential Radiologic Abnormalities
Lingual motion • Insufficient lip closure
• Tongue thrust
• Limited mandibular
movement
• Tongue weakness;
reduced oral sensitivity
• Tongue incoordination
• Reduced tongue elevation
• Contrast falls out of mouth
• Contrast pushed out of mouth
• Contrast stays on tongue or goes
into sulci
• Inadequate bolus formation
• Adherence to hard palate
• Piecemeal deglutition
• Prolonged oral transit time
Pharyngeal
swallow initiation
• Delayed onset
• Failure to initiate swallow
• Contrast in valleculae and pyriform
sinuses
• Contrast in pharyngeal recesses
Palatal pharyngeal
approximation,
pharyngeal
transport and
clearance; airway
invasion/laryngeal
closure
• Nasopharyngeal reflux/
pharyngonasal reflux
• Breathy or hoarse voice
• Gurgly voice quality
• Coughing or gagging
(silent aspiration
common)
• Insufficient or delayed velar
retraction and /or velar elevation
• Glottic incompetence or incomplete
laryngeal closure → penetration or
aspiration during swallow
• Contrasts in pharyngeal recesses
→ penetration or aspiration before
swallow
• Reduced pharyngeal motility →
penetration or aspiration after
swallow
• Residue in pharyngeal recesses
→ penetration or aspiration after
swallow
• Prolonged pharyngeal transit time
Esophageal entry
and clearance
• Delayed swallow
• Regurgitation or emesis
• Rumination
• Globus sensation (often
reported at level of
suprasternal notch)
• Cricopharyngeal dysfunction
• Obstruction, stricture
• Reduced esophageal motility
• Retrograde movement of contrast
in esophagus
• Gastroesophageal reflux

is the commonly used term to describe
contrast moving from pharynx over supe-
rior surface of the soft palate into the nasal
passage, or less frequently but likely more
accurate, pharyngonasal reflux or backflow
(Oestreich Dunbar, 1984).2
Nasopharyn-
geal reflux, can be observed (Figure 8–5).
Aspiration may be texture specific as noted
in Figure 8–4C. Residue in the valleculae
and along the tongue base is likely to result
from reduced tongue base retraction. When
residue is seen in the pyriform sinuses and
along the posterior pharyngeal wall, the
pharyngeal stripping wave is likely reduced.
The lack of base of tongue and posterior
pharyngeal wall approximation may be sec-
ondary to impaired pharyngeal strength or
motility (Figure 8–4B). Upper esophageal
function and dysfunction can be observed
(Case Study 1). Although esophageal tran-
sit time can be estimated or measured and
immediate gastroesophageal reflux can be
observed, VFSS is not the study to evaluate
these problems. Esophagram or UGI are the
examinations of choice.
When children present with clinical
signs of asymmetry, the posterior-anterior
(P-A) view may be helpful. The P-A view is
more difficult to obtain in infants and young
children in general and particularly in those
with poor head control. Further assessment
of the upper esophageal sphincter can also
be made with the P-A view (Figure 8–6A).
Figure 8–6B shows lateral view to compare
with the P-A view of the same infant. Care-
ful selection of patients who may benefit
from imaging in the P-A view is impor-
tant because each additional image during
the VFSS examination increases radiation
exposure.
Clinicians must make decisions about
the number of swallows needed for any
specific texture, remembering that a major
purpose is to define the pharyngeal swallow
physiology and not what happens with every
texture or consistency that a child takes.
Every additional swallow imaged increases
radiation exposure. Regardless of age, the
entire oral and pharyngeal regions of inter-
est can be observed in the lateral plane.
Esophageal transit is typically observed
with one bolus screened as it passes through
the esophagus to the lower esophageal
sphincter. Decisions about screening the
2

Pharyngonasal backflow may provide a more accurate description of bolus flow than the term nasopha-
ryngeal reflux. Nonetheless, the term nasopharyngeal reflux will be used throughout this book because it
is the more commonly recognized term.
Figure 8–5. Nasopharyngeal reflux in a
2-month-old infant with 22q11.2 deletion
syndrome (see black arrow).

351
Figure 8–6. A. Anteroposterior view of a 4-day-old infant
with upper esophageal spasm or narrowing (arrows) of upper
esophageal sphincter (UES); esophagus (E) and clavicles (C)
are labeled for orientation. B. Lateral view of narrowed upper
esophageal sphincter (black arrows), trachea (T), barium-
filled esophagus (E), clavicles (C), and posterior impression
of an incidental right subclavian artery (A + white arrow).
B
A

esophagus should be made on the basis of
the clinic/bedside assessment and whether
presenting questions have been or would
be better answered by an upper GI series.
Treatment recommendations differ depend-
ing on extent and type of impairments that
include whether aspiration occurs before,
during, and/or after swallows and also on
which textures. Recommendations also dif-
fer according to the status of pharyngeal and
esophageal motility.
Aspiration is one of the primary findings
of interest on the VFSS, but it is not the sole
purpose of the study. Modifications of bolus
size, texture, and temperature of food and
liquid used during the VFSS help clinicians
make recommendations for minimizing or
avoiding aspiration. In some instances, rec-
ommendations may include compensation
strategies for aspiration. Depending on the
child, trials of some of these modifications
may be useful prior to the VFSS, which can
then shorten the procedure. For example,
if children do not accept “real” liquids or
foods with specific textures, it is unlikely
that they will accept barium contrast that
simulates the rejected items.
Age and underlying condition may
influence whether specific textures are
aspirated (Weir, McMahon, Barry, Mas-
ters, Chang, 2009). Regardless of a child’s
age and underlying condition, liquids fre-
quently are handled less safely than other
boluses (Arvedson et al., 1994; Velayutham
et al., 2017). Some persons with neurologic
impairments are most efficient at handling
food that maintains a homogeneous consis-
tency (e.g., pudding), which resists separa-
tion into particles, remains discrete despite
changing shape as it is propelled through
the pharynx, and does not adhere to the
mucosa. Liquids frequently are handled
less safely than other boluses (Arvedson
et al., 1994). In contrast, persons with neu-
rologic conditions characterized by weak-
ness may exhibit residue with an increased
risk of aspiration because of difficult clear-
ing thicker contrasts from the mouth and
pharynx (Banno et al., 2017). Data are lack-
ing about the potential impact of cold ver-
sus warm temperatures, sour boluses, and
carbonation on swallowing physiology dur-
ing VFSS in children. Research is needed to
determine whether specific temperatures
or chemesthetic stimuli can induce adap-
tive physiologic changes in children with
dysphagia.
Differences in efficiency of swallowing
liquids can be noted whether liquid is pre-
sented via nipple, spoon, cup, or syringe.
Changes in head and neck position can be
observed as the child takes liquid in a vari-
ety of ways. Neck flexion (also known as
“chin-down posture” or chin tuck) has been
shown in adults to improve airway protec-
tion by causing a posterior shift of the ante-
rior pharyngeal structures and a narrowing
of the laryngeal entrance (Welch, Loge-
mann, Rademaker, Kahrilas, 1993). As a
cautionary note, neck flexion is contraindi-
cated in infants, particularly those born pre-
term, because it can cause airway obstruc-
tion (Thach Stark, 1979). Hyperextension
of the neck may interfere with swallowing
that is typically accomplished with the neck
at midline or in slight flexion. Nonetheless,
the possibility that a child uses neck hyper-
extension as a compensatory action for a
swallowing difficulty, particularly aspira-
tion, must be considered. It is important
to observe whether hyperextension of the
neck occurs unrelated to swallowing. Some
children hyperextend the neck to main-
tain airway patency because of neurologic
posturing or reflux. Recommendations for
changing head and neck position are made
on the basis of videofluoroscopic findings
along with clinical observations and reports
from caregivers. There is no one right posi-
tion for all children.
Clinicians may be able to develop ben-
eficial management strategies when the type

and severity of swallowing impairments on
videofluoroscopic studies are interpreted
within the context of the child’s underly-
ing diagnostic conditions and comorbidi-
ties, age, developmental status, and envi-
ronment (Arvedson Lefton-Greif, 2017;
Lefton-Greif McGrath-Morrow, 2007;
Martin-Harris et al., 2000). Infants and
children without sufficient cognitive levels
to comprehend and follow verbal directions
to cough, clear their throat, or hold their
breath and then swallow, will not be able to
benefit directly from interventions involv-
ing therapeutic maneuvers. However, they
may respond to modifications in feeding
routines or sensorimotor therapies. Vocal
quality is noted. Gurgly phonation during
and immediately after swallows may be an
indication of residue spilling into the laryn-
geal vestibule, which relates to heightened
risk for aspiration. A breathy or hoarse voice
quality raises suspicions for incomplete
vocal fold closure and a heightened risk for
aspiration, and should prompt evaluation
by an otolaryngologist (Banno et al., 2017).
Advantages and
Disadvantages of VFSS
Advantages and disadvantages of VFSS are
shown in Table 8–4. As discussed, the best
results are gained when patient cooperation
is maximized, the study is carefully planned
based on clinical examination and under
lying conditions, and equipment and per-
sonnel are appropriate for performance of
the study.
Interpretation:
Pearls and Pitfalls
Accuracy of interpretation of findings and
good intra- and interrater reliability are crit-
ical to the clinical utility of the VFSS. Termi-
nology should be precise and standardized.
The importance of standardized procedures
and extensive training are also critical to
improve accuracy of identification of find-
ings (Henderson et al., 2016; Lefton-Greif
et al., 2018; Nordin et al., 2017). Typically,
VFSS images are reviewed jointly by the
SLP and radiologist for interpretation of
the results. A team of specialists involved in
the child’s care can be very helpful in mak-
ing comprehensive management decisions
on the basis of VFSS findings and multiple
other factors when multiple systems are
involved in complex ways (see Figure 8–1).
The detailed report of findings is usually
prepared by the SLP. Findings from VFSS
can be discussed from a variety of view-
points. This section summarizes swallowing
function in relation to abnormal radiologic
findings (see Table 8–3). Bolus formation,
or oral preparation, is discussed briefly for
completeness, but the VFSS is not carried
out to define bolus formation/oral prepara-
tory function, which can be imaged better
with US and described well in the clinical
evaluation of swallowing and feeding.
Efficient intake requires effective lip
closure and bolus formation—if these are
lacking, liquid and food dribble out of
the mouth. Tongue and buccal hypotonia,
uncoordinated tongue movement, and
reduced oral sensation may interfere with
the child’s ability to form a bolus. Limited
or inefficient tongue and mandibular move-
ments may result in piecemeal deglutition.
The ability to hold material and form a bolus
of any texture in preparation for the poste-
rior tongue propulsion may be affected. As
food or liquid moves throughout the oral
cavity, VFSS images can show barium con-
trast in the frontal and lateral sulci, adher-
ence to the hard palate, piecemeal deliv-
ery of boluses related to movement to the
posterior tongue, and food or liquid falling
into the pharyngeal recesses before initia-
tion of a pharyngeal swallow. Thus, a child

may aspirate as material gets into the open
airway before a swallow is produced. Thin
liquids pose the greatest risk of preswallow
aspiration.
In infants and young children, abnormal
delay in initiation of swallowing increases
the risk for aspiration consequences. Adults
and older children, as well as infants and
young children, often collect the bolus in
the valleculae before onset of the pharyn-
geal swallow (Zancan, Luchesi, Mituuti,
Furkim, 2017). Prolonged holding (stasis) of
a bolus in the pharynx before the initiation
of a swallow poses an increased risk of aspi-
ration during the next inhalation. Infants in
whom the cough reflex is less developed or
blunted have a high probability for silent
aspiration (Thach, 2001).
Delay in swallow initiation needs to be
differentiated from a cricopharyngeal dis-
order or delayed opening of the cricopha-
ryngeus, a primary muscle in the upper
Table 8–4. Advantages and Disadvantages of Videofluoroscopic Swallow Study (VFSS)
Advantages Visualizes swallowing during bolus passage through the oral,
pharyngeal, and upper esophageal structures
Timing of initiation of swallow and coordination with oropharyngeal
structures is seen
Residue in pharyngeal recesses and pyriform sinuses is seen
Physiologic deficits can be correlated with timing and an approximate
amount of aspirated material
Response to aspiration can be seen and heard with simultaneous
recording of audio signals
Swallowing impairment can be correlated with degree of aspiration
Oral and pharyngeal transit times can be calculated
Available in most medical institutions
Disadvantages Uses ionizing radiation; therefore, studies must be short
Requires patient cooperation
Equipment for positioning can be cumbersome and limits positioning
options
Patient must be taken to radiology suite (portable units in some places)
Requires trained personnel
Requires patient cooperation in an x-ray suite with specialized seating
equipment
Requires ingestion of barium contrast material, which alters the taste
and texture of liquids and foods
Captures a brief view of the swallowing function that necessitates
clinical correlation to address the potential of underestimating risk of
aspiration during the study
Standardized tools for objective reading and interpretation of images
are not readily available for infants and young children at this time
Source: Adapted from Arvedson Brodsky (2002);Arvedson Lefton-Greif (2017);Lefton-Greif et al.(2018).

esophageal sphincter (UES) or pharyngo-
esophageal sphincter (PES). The absence
of UES opening is more likely to relate to
neurologic control of brain-stem function
than to a true structural disorder. Excep-
tions do occur, and clinicians must be able
to differentiate the types.
Prolonged pharyngeal transit time and
reduced pharyngeal motility, in particular,
place a child at high risk for aspiration,
even if tongue action is adequate in bolus
formation and oral transit. Nonetheless,
the vast majority of children with neuro-
logic impairments show at least some oral
preparatory and oral function problems
along with significant pharyngeal deficits.
Nasopharyngeal reflux may indicate dys-
functional velopharyngeal closure or tim-
ing problems. Incoordination of pharyngeal
phase and reduced pharyngeal motility
often result in material remaining in the val-
leculae and pyriform sinuses after the swal-
low (residue), more commonly seen with
thicker boluses. Aspiration of the residue
occurs when material spills into the open
airway after the swallow. Children with neu-
romuscular weakness associated with spinal
and bulbar muscular atrophy or other diag-
nostic conditions characterized by reduced
pharyngeal motility are more likely to have
postswallow residue that results in aspira-
tion of thick textures (Banno et al., 2017;
Griggs, Jones, Lee, 1989).
Unilateral signs and symptoms are less
common in children than in adults. Chil-
dren who have had strokes, brain-stem
tumors, congenital conditions, or surgeries
involving one side of the head/neck struc-
tures may show unilateral signs. In those
circumstances, a P-A view may be helpful.
Although reduced laryngeal elevation is rel-
atively uncommon in young infants as the
larynx is positioned high in the neck, vocal
fold closure may be incomplete or incoor-
dinated and result in aspiration most com-
monly seen during swallows of thin liquid.
In infants and young children, aspiration
events are typically silent (Velayutham et al.,
2017), but in some instances observable
actions could include coughing, gagging,
or breathy and hoarse voice quality. Clini-
cians heighten their index of suspicion as
the number of health-related variables and
observable signs increase.
Cricopharyngeal dysfunction is rela-
tively rare in infants and children, but cri-
copharyngeal spasm, pharyngoesophageal
spasm, or cricopharyngeal achalasia may
occur. These relatively isolated findings are
likely to be a marker for broader disabilities
that may emerge as the child grows. Fig-
ure 8–6 shows a 4-day-old infant with fluo-
roscopic findings consistent with an upper
esophageal swallow deficit. Children with
Arnold-Chiari malformations are reported
to have neurogenic dysphagia with a combi-
nation of diffuse pharyngoesophageal dys-
motility, cricopharyngeal achalasia, nasal
regurgitation, tracheal aspiration, and gas-
troesophageal reflux (Fuller et al., 2016; Liu
Ulualp, 2015; Putnam et al., 1992).
The esophageal phase of the swallow can
be impaired because of a number of neu-
rologic conditions or structural anomalies.
Some of the more common possibilities in
infants and children include reduced esoph-
ageal motility or dysmotility, obstruction or
stricture, or esophagitis. Gastroesophageal
reflux frequently contributes to esophagi-
tis and worsens swallowing function (dis-
cussed in detail in Chapter 5).
Whereas a trace amount of barium that
is aspirated during diagnostic procedures
does not appear to be clinically significant,
aspiration of larger amounts of barium is
not benign and should be avoided during a
VFSS or any other procedure (Jackson et al.,
2014). Careful planning prior to the VFSS
may avoid some adverse aspiration-induced
problems. If there are concerns about the

amount of aspiration or the child’s response
to the aspiration, appropriate medical inter-
vention is essential. Suctioning capabilities
and appropriate personnel should be avail-
able for infants and children with tracheos-
tomies and those with other major respira-
tory concerns.
Review and Report of Findings
Digitized images of the VFSS are reviewed
by the SLP with caregivers following the
procedure. The SLP makes recommenda-
tions and writes the report reflecting the
team analysis (radiologist and SLP; at times
may involve other physicians, including a
developmental pediatrician, neurologist,
pulmonologist, gastroenterologist, or oto-
laryngologist). Caregivers find the review
of VFSS images particularly helpful when
a child has residue following swallows or
aspirates silently. This review lets them see
material in the airway. The audio lets them
hear that the child did not cough. Real-time
and slow-motion reviews provide caregiv-
ers with vivid images that aid their under-
standing, which in turn helps them to follow
through with recommendations.
Caregivers are alerted that constipation
may be a possible side effect, although low
probability, of the barium sulfate ingestion.
Thus, recommendations are made by physi-
cians for additional fluid intake and, in some
instances, a laxative or suppository. Because
the amount of barium contrast material is
relatively small, constipation problems are
not common. Allergic reactions are rare
but should be mentioned. Caregivers are
encouraged to consult with the child’s pri-
mary physician in the rare instances when
side effects may occur.
Children with complex problems may
undergo team consultation and interdis-
ciplinary recommendations. The digitized
images can be available for review during
follow-up clinic visits or other conferences.
Findings from any particular diagnostic test
are useful only when considered within the
total context of the child. When caregivers
fully understand and appreciate the results,
they can incorporate recommendations into
the child’s daily activities.
Older children, particularly those with
higher cognitive function, find the feed-
back helpful when the images are used in
the treatment process. Images may be help-
ful for caregivers of young patients or those
with significant cognitive deficits. In addi-
tion, examples of images that demonstrate
normal swallows and abnormal swallows
from various etiologies can be helpful in
training. This training should be useful for
graduate students as they prepare to become
professionals in this high-risk area of patient
care and for professionals already involved
with pediatric swallowing and feeding.
Findings must be reported to the refer-
ral source and other health care provid-
ers involved with the child. In addition
to providing objective findings from the
study, clinicians must provide information
regarding whether the findings are consis-
tent with the child’s underlying condition
or presenting problems, whether the child
cooperated during the study, and any other
factors that may influence the management
of the child. Reporting just the presence
or absence of penetration or aspiration is
insufficient for the development of appro-
priate management.
Severity Classification
Classification of severity of swallowing
impairments in children would be helpful
to compare findings among evaluators in
multiple institutions, to monitor changes
over time through various intervention
and management strategies, to exchange

information, and to measure outcomes.
Recent reports show that standardization of
VFSS procedures and the reading of images
obtained from adults and children, hold
promise for advancing the clinical utility
of the evaluations one without increasing
radiation exposure (Bonilha et al., 2013;
Henderson et al., 2016 ; Stoeckli, Huisman,
Seifert, Martin-Harris, 2003). Further
research is needed to determine whether
and to what degree standardization may
have a comparable impact for infants and
young children undergoing VFSS proce-
dures (Lefton-Greif et al., 2018; Nordin
et al., 2017).
Themostbasicquestioniswhetheraper-
son is safe for oral feeding of any texture(s).
The severity of swallowing impairment will
provide critical information. However, the
answer(s) to this question are complex and
dependent on multiple factors including
those related to the child, the timing/age
and its relation to growth and vulnerability
to the consequences of dysphagia, and envi-
ronmental/social factors (see Figure 8–1).
The Penetration-Aspiration (PenAsp),
Scale, an 8-point scale, was developed spe-
cifically to quantify selected penetration and
aspiration events observed during VFSS in
adults (Rosenbek, Robbins, Roecker, Coyle,
Wood, 1996). This type of scale has poten-
tial for improved precision in describing
the accidental loss of food or liquid into the
airway while eating or drinking. Although
penetration has been observed in more than
11% of normal adults participating in VFSS,
comparable findings have been associated
with significantly more cases of pneumonia
in children (Allen, White, Leonard, Belaf-
sky, 2010; Gurberg, Birnbaum, Daniel,
2015). Thus far, data validating the clinical
significance of the PenAsp scale in infants
and children are not available.
There is limited research on the appli-
cation of videofluoroscopic findings to
management decisions for the pediatric
population. Findings that represent a lim-
ited sample in a brief period of time on
one occasion must be placed into context
with history and clinical findings. Develop-
ment of salient clinical outcome measures is
urgently needed.
Repeat VFSS Studies
A frequently asked question is, “When do
you repeat a VFSS?” The simple answer is “as
seldom as possible.” The same criteria that
were used in decisions for an initial study
are used for any follow-up study. A signifi-
cant change in health status, recurrence of
previous signs and symptoms, prior history
of silent aspiration, or failure to grow may
indicate possible need to change diet tex-
tures or some other aspect of intervention.
Swallow status is one of the variables, which
then must be reevaluated. Improved oral
sensorimotor functioning in children with
profound cognitive deficits and neurologic
impairments has not proven to correlate
with improved pharyngeal function. VFSS
findings are useful when concerns relate
to pharyngeal swallow function and the
need to define status for possible changes
in management.
Management options may include
changes in diet textures; positioning and
seating; specialized feeding tools and tech-
niques; behavioral modification; direct
oral sensorimotor practice; sensory-based
approaches; and/or specific instructions
related to coughing, breathing, or timing of
the swallow. Alternate methods of nonoral
feeding may need to be considered, with
plans for oral sensorimotor stimulation
with or without food to be included in the
practice. These management options are
discussed in detail in Chapter 9.

Ultrasound Imaging
of Swallowing
Ultrasound Definitions
and Procedures
US is defined as sound propagated at fre-
quencies above those audible to the human
ear, that is, over 20 kHz. The frequencies
used for diagnostic imaging range between
2 and 10 MHz, a 1,000-fold increase above
the audible range (Sonies, 1991). During US
imaging, a transducer is used both to gener-
ate sound waves and to receive ultrasound
echoes. These echoes are then electronically
converted into computer-generated images.
An ultrasound beam, directed into a soft tis-
sue medium, vibrates tissue particles in that
medium. Body tissues (such as fat, muscle,
and fascia) and fluids (blood, cerebral spinal
fluid, and water) have different densities and
reflect echoes back to the transducer at dif-
ferent intensities, making different tissues
or other substances distinguishable from
each other at their interface. In real time,
US imaging displays movement of anatomic
structures. Doppler is a type of ultrasound
that can detect and measure fluid flow and
has been used to diagnose many medical
conditions (e.g., blood clots, heart valve
defects and congenital heart disease, and
oligohydramnios [insufficient volume of
amniotic fluid during pregnancy]).
Ultrasound with Infant
Breast- and Bottle-Feeding
US has been used to define prenatal predic-
tors of postnatal feeding skills and oral skill
maturation (Miller, Macedonia, Sonies,
2006; Miller, Sonies, Macedonia, 2003).
Doppler images can augment the typical
US images in the fetus by detecting amni-
otic fluid flow and respiratory function
(Macedonia, Miller, Sonies, 2002; Miller
et al., 2006; Miller et al., 2003). US technol-
ogy has been applied to visualize temporal
relationships between movement patterns
of oral and pharyngeal structures in infants
(Bosma, Hepburn, Josell, Baker, 1990;
Smith, Erenberg, Nowak Franken, 1985;
Weber, Woolridge, Baum, 1986) as well
as in older children and adults (Fanucci,
Cerro, Ietto, Brancaleone, Berardi, 1994;
Shawker, Sonies, Hall, Baum, 1983, 1984;
Sonies, 1990; Stone Shawker, 1986; Yang,
Loveday, Metreweli, Sullivan, 1997).
During the postnatal periods, US is
most helpful in describing bolus formation
and oral transit, particularly in bottle- or
breastfed infants (Bosma et al., 1990; Ged-
des Sakalidis, 2016; McClellan, Sakalidis,
Hepworth, Hartmann, Geddes, 2010). In
addition, US imaging has the potential to
track the maturation of sucking patterns in
the early postpartum period and distinguish
between tongue actions associated with
nutritive and nonnutritive sucking (Sakali-
dis et al., 2013).
US imaging is particularly useful when
there is concern for an oral problem during
breastfeeding. Both mother and infant can
be observed for extended time periods to
define the oral function without concern for
radiation exposure in contrast to VFSS (as
previously discussed). For example, when a
breastfeeding infant fails to initiate sucking
readily or shows incoordinated sucking, it
is possible to observe whether the difficulty
is with the infant’s suck–swallow patterns or
the maternal milk supply (McClellan et al.,
2010; Smith et al., 1985). Muscle interac-
tions and nipple deformation must be
functional for successful oral feeding; these
are both clearly seen with US (Sakalidis
et al., 2013).

Ultrasound for Older Children
With Pharyngeal Concerns
Whereas the oral function is visualized well
using US, disordered oropharyngeal swal-
lows are less well imaged, particularly for
older children and adults when cartilages in
the larynx have calcified and interfere with
the transmission of the US waves. Impor-
tantly, oral preparatory and oral transit are
under voluntary control and therefore have
the greatest potential to be altered with oral
sensorimotor and/or other behavior-based
therapies.
Advantages and Disadvantages
of Ultrasound
The advantages and disadvantages of US are
found in Table 8–5. Although US scans are
noninvasive, one of its primary limitations
is that sound echoes do not pass through
bone. Therefore, its utility is limited to oral
functions, including bolus formation and
transfer. Currently, US is not used routinely
for assessment of swallowing because of
limitations in viewing the structure of the
pharynx and the need for extensive train-
ing to become proficient in carrying out
Table 8–5. Advantages and Disadvantages of Ultrasound
Advantages Excellent soft tissue delineation in oral cavity
Provides dynamic views of oral preparatory function and oral transit
Multiplanar—sagittal, coronal, and, in infants, transverse
Provides images of swallow structures at rest and during bolus
movement
Captures tongue, palate, and hyoid activity for detection of laryngeal
elevation with initiation of pharyngeal swallow
No radiation exposure
Swallows can be sampled repeatedly and for prolonged periods of time
Requires no contrast, uses “real” food or liquid
Equipment is available at major medical centers
Body positioning for patient and mother may not be problematic
Fluid flow can be detected when standard ultrasound is paired with
Doppler
Disadvantages Limited to oral preparatory phase and oral phase swallowing in most
instances
Cannot detect laryngeal penetration or aspiration directly after
calcification of the cartilages comprising the upper aerodigestive tract
Structural landmarks are difficult to identify and may need to be
identified by markers
Laryngeal structures cast shadows that obscure view of the airway
Availability of trained personnel may be limited
Widespread clinical use is limited because of a shortage of data and
trained personnel

the examination and in interpreting find-
ings (Arvedson Lefton-Greif, 2017). As
sophistication in the diagnosis and treat-
ment of swallowing and feeding disorders
continues to evolve, US may emerge as a
more widely used imaging tool for clinic
purposes, especially in infants. Coordina-
tion and comparison with other imaging
procedures, such as VFSS and FEES, will
be necessary to gain maximal information
from this imaging modality.
Case Studies
Case Study 1
History and Presentation
“Michael” was delivered at term following
an uncomplicated pregnancy. Meconium
aspiration was noted and required vigor-
ous suctioning, but not intubation. He
was transferred to the regional neonatal
intensive care unit (NICU) on Day 2 of life
because of cyanosis during oral feeding. He
coughed and gagged with feeding. A weak,
breathy, and hoarse cry was noted.
Initial Examination
He had normal vocal fold function via flex-
ible fiberoptic nasopharyngoscopy (FFNL).
Direct rigid laryngoscopy revealed that ary-
tenoids were characterized by mild edema.
A VFSS revealed nasopharyngeal reflux and
insufficient opening of the upper esopha-
geal sphincter. A focal spasm was seen in the
esophagus just below the cricopharyngeal
sphincter (see Figure 8–6). No aspiration
was evident on the initial VFSS. Physicians
recommended no direct medical or surgical
intervention for at least several weeks but
close monitoring because time and growth
may allow for spontaneous resolution.
An NG tube was used for feeding to
meet nutrition needs. Oral sensorimotor
assessment revealed normal nonnutritive
sucking and oral sensation. This infant did
not tolerate more than a drop of 5% glucose
solution or water on the tip of his mother’s
finger or on a pacifier without coughing,
gagging, and becoming cyanotic. Addi-
tional diagnostic workup included com-
puted tomographic (CT) scan of the head,
which was normal. No liquid feedings could
be attempted in the neonatal period. He was
discharged to home on NG tube feedings to
be reevaluated several weeks later.
Postdischarge From NICU
Parents continued to provide oral stimu-
lation for nonnutritive sucking, and very
small amounts of liquid for test and prac-
tice purposes. After a few weeks, flavored
liquids were presented in small amounts
via syringe for practice with close monitor-
ing to minimize stress. Esophageal dilata-
tion occurred at about 4 months of age. He
gradually increased volumes with oral feed-
ing and was a total oral feeder by 6 months.
Long-Term Follow-Up
Swallowing and Speech
Continued developmental examinations
revealed hypotonia, which became more
evident as he got older. Speech was charac-
terized by flaccid dysarthria and hyperna-
sality due to velopharyngeal insufficiency.
He continued to have difficulty swallowing,
noted with gulping and multiple swallows
needed for thin liquids. Follow-up VFSS at
age 3 years revealed similar upper esopha-
geal deficits and nasopharyngeal reflux,
although he was a functional oral feeder
with no clinical presentations of aspiration.
Multiview videofluoroscopic speech study
also revealed velopharyngeal insufficiency.

“Michael” needed coaxing and preparation
for FFNL in voice clinic with otolaryngolo-
gist and SLP. Findings were consistent with
perceptual speech motor findings and the
fluoroscopy. In addition, on endoscopy, a
prominent pulsating carotid artery was evi-
dent. A diagnosis of 22q11.2 deletion syn-
drome was confirmed by genetics workup.
Comment
This child is an example of the importance
of delineating pharyngeal and esophageal
function of swallowing to make appro-
priate oral sensorimotor and swallow-
ing recommendations. There is no direct
oral sensorimotor treatment that can alter
esophageal functioning. Nonnutritive suck-
ing experience can be provided, with con-
tinued assessment to monitor change and
potential for oral feeding. The importance
of a thorough diagnostic workup with feed-
ing problems in the neonatal period cannot
be overemphasized. Parents need as much
objective information as possible when they
cannot feed their newborn in typical ways.
Follow-up over time is also vital. Significant
swallowing and feeding problems in the
neonatal period are often markers for more
global developmental problems to become
evident over time. Although ideally the
genetics diagnosis should have been made
earlier, in this instance, the parents stated
that the diagnosis would have been harder
to accept had it been made earlier. Sensi-
tivity and communication with parents are
usually as important as actual findings.
Case Study 2
History and Initial Presentation
“Jaedyn” is a 2-month-old female born at
29 weeks’ gestation following a pregnancy
complicated by cocaine exposure. She had
been given opportunities for nonnutritive
sucking (NNS) via pacifier until medically
stable for readiness to be seen to evaluate
for oral feeding potential. She was seen at
35 weeks’ corrected age for a bedside feed-
ing/swallowing evaluation before advancing
oral feeding and to help guide short- and
long-term feeding management decisions.
Her NICU course was characterized by
sequelae associated with her prematurity
that included chronic lung disease, Grade II
intraventricular hemorrhage (IVH), feed-
ing intolerance (intermittent desaturation
events with gavage feeding), and respiratory
distress with previous oral feeding attempts.
Limited bottle feedings of 10 to 15 ml con-
tinued with supplemental NG tube feedings.
Reevaluation Over Time
On reevaluation, she presented with a
strong NNS and variable incoordination
of suck/swallow/breathe sequencing with
efforts to increase the volume of oral intake.
VFSS was performed to evaluate for swal-
lowing impairments, including aspiration
risks that could explain her feeding prob-
lems. The VFSS demonstrated no pharyn-
geal phase deficits, and no penetration or
aspiration; there was trace and intermittent
nasopharyngeal reflux, which is considered
within normal limits for young infants. It is
also possible that the NG tube added resis-
tance to nasal airflow. The VFSS was helpful
because findings supported her readiness
to proceed gradually with oral feeding. She
became a full oral feeder within 10 days and
was discharged to a foster family.
Comment
This infant demonstrates the importance of
defining pharyngeal physiology when there
are clinical signs of respiratory concerns

that may interfere with safe oral feeding. She
also demonstrates the importance of under-
standing the impact of “normal” findings
(e.g., nasopharyngeal reflux) on feeding.
Nurses implemented guidelines for maxi-
mizing efficiency of nipple-feeding with
greater confidence once they had the VFSS
findings. The foster parents were trained
to carry out feedings with the best tech-
niques for posture, position, monitoring of
flow rate, and pacing as needed. This infant
grew well over the next several months of
life and made good developmental progress
as anticipated for infants with Grade II IVH.
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369
Management of Swallowing
Summary
Decision-making for management of infants
and children with swallowing and feeding
disorders (Goday et al., 2019) is based on
knowledge of and goals for adequate nutri-
tion and gastrointestinal function, stable
pulmonary function, and developmentally
appropriate oral sensorimotor and feed-
ing skills. Medical, surgical, and nutrition
considerations are all critical for identify-
ing optimal management strategies and are
detailed in other chapters. Ideally, all man-
agement decision-making occurs within the
context of maximal participation of children
in the social and communication activities
associated with family mealtimes. Some
children are total oral feeders. When chil-
dren cannot meet their nutritional needs
safely by oral feeding alone, supplemental
nutrition routes are needed. Nonoral feed-
ing should not be viewed as a failure or last
resort, but should be approached as a means
or “tool” for maximizing safety, growth, and
development. Underlying condition(s),
chronologic and developmental age of the
child, social and environmental arena,
and psychologic and behavioral factors all
impact on management recommendations
that include specific therapeutic interven-
tions. This chapter delineates principles
underlying swallowing and feeding func-
tions with specific focuses on oral senso-
rimotor and posture/positioning function.
Introduction
Optimal management is critical for all
infants and children with swallowing and
feeding disorders and requires:
n establishment of adequate nutrition and
gastrointestinal function,
n maintenance of stable respiratory func-
tion and pulmonary health, and
n maximal participation in activities
that are dependent on swallowing and
feeding skills.
Although the strategies for achiev-
ing these goals may change over time, the
basic goals are invariant. The importance of
interdisciplinary communication and team
decision-making is emphasized repeatedly
throughout this book, and its application is
further elaborated in this chapter as essen-
tial to the attainment of the most favorable
outcomes. The selection of strategies is
often discipline specific, and these strategies
9

may include, but are not limited to, changes
in the route of nutrition and hydration,
adjustments in nutrition guidelines, dietary
and bolus modifications, utensil changes,
behavioral therapies, and oral sensorimo-
tor interventions.
This discussion of management focuses
on oral sensorimotor interventions and
position/postural adjustments that are
addressed in early intervention programs,
schools, and rehabilitation programs.
Regardless of setting, management deci-
sion-making and adjustments in therapeu-
tic interventions occur during every evalu-
ation/examination, just as all intervention
sessions include evaluation of status (Lef-
ton-Greif Arvedson, 2016). Thus, it is not
possible to separate evaluation and manage-
ment completely given the integrated func-
tions within each infant and child.
Although the field of swallowing and
feeding disorders in children is still in its
infancy, evidence-based practice is encour-
aged for all clinicians involved in the assess-
ment and management of these high-risk
infants and children. Limited studies are
available that focus on the many situations
encountered in this heterogeneous and
challenging population. In 1993, Archie
Cochrane’s call for up-to-date, systematic
reviews of all relevant randomized con-
trolled trials of health care developed and
gave rise to the Cochrane Collaboration
(https://www.cochrane.org/). Systematic
reviews of interventions have become the
focus of an international group of clinicians,
methodologists, and consumers contribut-
ing to the work of this collaboration.1
At the
time of this writing, Cochrane reviews that
address multiple areas involving swallow-
ing and feeding in children are accessible.
Clinicians are urged to collect data in their
own settings and to participate in multi-
center clinical trials whenever possible. It is
through careful observation, reporting, and
collaboration that advances in both diagno-
sis and treatment can be made.
This chapter provides an overview of
general management principles, followed
by oral sensorimotor interventions and
position/postural adjustments for these
high-risk children and will include the best
available evidence for any and all therapies.
Case reports demonstrate the complexities
of feeding in individual children who are
best served by an interdisciplinary team.
Principles for Decision-
Making With Sensorimotor
Learning Principles
and Neural Plasticity
Sensorimotor learning principles are fun-
damental to management considerations
for infants and children with essentially
all swallowing and feeding disorders (e.g.,
Sheppard, 2008). These concepts apply most
directly to infants and children with under-
lying neurologic deficits. Patients with oro-
pharyngeal dysphagia typically demonstrate
signs of neurologic deficits, even when neu-
roimaging does not provide a clear underly-
ing etiology. Neural plasticity appears to be
the basis for learning in the intact brain as
well as in relearning in the damaged brain
(e.g., Kleim Jones, 2008). Their review
of 10 principles of experience-dependent
neural plasticity provides considerations for
applications of those principles to the dam-
aged brain (Table 9–1). Studies of neurobi-
1
Cochrane Database of Systematic Reviews. Available from BMJ Publishing Group, P.O. Box 295, London
WC1H 9TE, UK. Tel: +44 (0)20 7383 6185/6245; Fax +44 (0)20 7383 6662. Reviews are published on
computer disk and CD, on the Internet, and in a variety of other forms.

9. Management of Swallowing and Feeding Disorders 371
ological phenomenon related to functional
recovery may provide a basis for identifi-
cation of fundamental principles that may
help to guide optimization of interven-
tion. Every principle may not appear to
the reader to have direct application to oral
feeding. They all are relevant at one or more
levels described in Table 9–1.
General Principles for
All Interventions
Morethanacenturybeforethetermevidence-
based medicine (EBM) was coined, Florence
Nightingale was using the basic data collec-
tion and statistic inquiry elements that are
Table 9–1. Experience-Dependent Neural Plasticity Applicable to Swallowing and Feeding
Principle Descriptions for Consideration in Infants and Children
Use it or
lose it
Lack of use can lead to functional degradation; learning is essential
component of brain adaptation, must be carried out in ways to be
adaptive, not maladaptive.
Use it and
improve it
Training to drive specific brain function with goal to leading to an
enhancement of that function.
Specificity Experiences during training provide the basis for the nature of the
plasticity; skill acquisition is associated with changes in activation in
motor cortex; specific forms of neural plasticity and associated behavioral
changes are dependent on specific kinds of experience.
Repetition
matters
Repetition of newly learned (or relearned) skills/behavior may be needed
to induce lasting neural changes. Repetition may be critical for habilitation
and rehabilitation—in order to reach a level of function for individuals to
attain carry over and make additional functional gains.
Intensity
matters
Intensity of stimulation or training can affect induction of neural plasticity.
Caution: possible to over train leading to negative consequences.
Time matters Specific times for interventions may lead to better outcomes than other
times (not clearly defined—consider critical and sensitive periods for
leaning). Early intervention often advocated. For some, delayed intense
intervention may result in better outcomes.
Salience
matters
Important tasks that are functional are more likely to be perceived and
attended to—more likely to be learned.
Age matters Timing and sequence of developmental processes of the central nervous
system during infancy and childhood provide unique opportunities for
learning.
Transference Learning in one type of training situation may facilitate learning of other
similar behaviors.
Interference Learning in one type of training situation may interfere with learning of
other behaviors.
Source: Adapted from Kleim, J. A., Jones, T. A. (2008). Principles of experience-dependent neural plastic-
ity: Implications for rehabilitation after brain damage. Journal of Speech, Language and Hearing Research,
51(1), S225–S239.

fundamental to EBM to achieve improve-
ments in patient care and outcomes (Weiner
Sauter, 2003). In 1996, Sackett and col-
leagues defined EBM as the “conscientious,
explicit, and judicious use of the current
best evidence in making decisions about the
care of individual patients” (Sackett, Rosen-
berg, Gray, Haynes, Richardson, 1996).
Over time, the tenets of EBM have been
adopted by many professionals, who refer
to it as evidence-based practice (EBP). A
primary principle of EBP is to offer patients
treatments that do more good than harm
and that are worth the efforts and cost of
using them. The American Speech-Lan-
guage-Hearing Association (ASHA) web-
site operationalized the definition of EBP
as “the integration of: (a) clinical expertise/
expert opinion, (b) external scientific evi-
dence, and (c) client/patient/caregiver per-
spectives to provide high-quality services
reflecting the interests, values, needs, and
choices of the individuals we serve” (https://
www.asha.org/Research/EBP/Introduc-
tion-to-Evidence-Based-Practice/; also
see: https://www.asha.org/Research/EBP/
Framing-the-Clinical-Question/).
Clinically, EBP is an indispensable safe-
guard against pseudoscience and poten-
tially the use of harmful assessment and
treatment methods (Lee Hunsley, 2015).
Importantly, even interventions that appear
to be plausible can be ineffective or exert
iatrogenic consequences (Lee Hunsley,
2015), and all interventions are associ-
ated with costs (e.g., time, money, and/or
energy). Unfortunately, objective data sup-
porting management in pediatric swallow-
ing and feeding disorders are limited and
often devoid of information that identifies
efficacious treatments. Nonetheless, clini-
cians are encouraged to review information
pertinent to their patients from credible
sources including but not limited to consen-
sus statements, white papers, and trustwor-
thy websites (e.g., PubMed [https://www
.ncbi.nlm.nih.gov/pubmed/] and OMIM
[https://www.ncbi.nlm.nih.gov/omim/]).
Frameworks for Management
Decision-Making
World Health Organization’s
International Classification
of Functioning,
Disability, and Health
The International Classification of Func-
tioning, Disability, and Health (ICF) model
is the World Health Organization’s (WHO)
approved classification of health and health-
related domains, which has shifted assess-
ment and treatment paradigms from focus-
ing on impairments to focusing on function
in the broad sense of participation (see
Chapters 1 and 7). As a bio-psychosocial
model, the ICF includes body functions/
structures, activities, participation, and
environmental and personal (social) factors.
Although all children with swallowing and
feeding disorders may exhibit a common
core set of factors, individual profiles (e.g.,
the ability to participate in meals at home,
in school, or in a restaurant) are likely to
be dependent on features unique to the
affected person (e.g., underlying diagnostic
condition, age/developmental status, and
severity of the dysphagia) (Schiariti, Mahdi,
Bolte, 2018). At this time, ICF-based tools
that provide

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